lupus panniculitis

systemic lupus erythematosus, abbreviatedas sle. this is a systemic disease, which means thatit can affect the whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, the vascular system, and the list goes on. lupus means wolf. erythematosus means reddening of the skin. why did i show a red butterfly? because a very typical sign of sle is a butterflyshaped reddening of the skin. the way i remember this disease, is to imaginea wolf that attacks a woman, and why a woman?

because 9 out 10 patients are women. so the wolf bites the face of the woman causinga butterfly shaped erythema on her face. then the wolf continues to bite all her organsone by one, because this disease is a systemic disease, which means that it can affect allthe organs systemically. of course this disease has nothing to do witha wolf. but sle is an autoimmune disease, which meansthat this woman called emma have an immune system that attacks her own body, insteadof only attacking bacterias, viruses and parasites. so emma have an immune system that acts asa wolf inside her.

i will show you how we can find out if emmareally has sle by diagnosing her. and then we will look at what type of medicationswe can give her. i want you to remember at least one thingfrom this presentation. that is systemic lupus international collaboratingclinics, abbreviated as slicc. slicc is a criterion that can help in thediagnosis of sle. slicc contains clinical criteria, immunologiccriteria and biopsy criteria. we need more than 4 criteria with at least1 clinical and 1 immunologic. or we need 1 biopsy criteria and 1 immunologiccriteria.

so what does these 3 words mean? clinical, immunologic and biopsy? the synonym for clinic, is hospital. so clinical criteria stand for the symptoms,signs and lab values of a patient in the hospital. immunology deals with the immune system thatprotects you from bacterias, viruses, and immunologic criteria are usually lab teststhat detect any problem with the immune system. biopsy means that a doctor cuts out a pieceof an organ. for example, a small piece of kidney can becut out, and then the doctor puts this piece of kidney in a microscope and looks for diseases.

clinical criteria can be divided into alopecia,which means hairloss. skin problemsulcers heart diseaseslung diseases kidney problemsjoint pain problems with the nervous systemand blood diseases. skin problems can be grouped under the namecutaneous lupus erythematosus. we can divide these skin diseases into acutesubacute and chronic, based on the timing of the diseases. acute diseases are usually those that happensuddenly with a short duration.

chronic diseases are ongoing for a long time. whereas subacute means those diseases thatare not yet chronic but has passed the acute phase. a way to remember these timing are to memorize2 numbers. 1 and 3. why? because the duration of acute diseases areusually less than 1 month, subacute are 1 to 3 months, and chronic are more than 3 monthsin duration. so which are the acute type of skin problems?

butterfly erythema, which is a reddening ofthe face in a butterfly shape. it usually affects the cheeks or also calledmalar region, and therefore it is sometimes called malar erythema. it does not affect the nasolabial region. there can also be bullous lesions which areblisters that can be a mix of small grouped vesicles to large tense bullae. bullae are elevating the skin and are filledwith fluid. toxic epidermal necrolysis-like sle, is adetachment of the epidermis, the upperlayer of the skin, resulting in exfoliation, meaningthat the upperlayer just falls off.

this is very dangerous, because the skin isthe organ that protects against infections. so if you loose a large part of your skin,then sepsis can happen, which means that bacterias easily invade your vascular system, sincethere is no skin to protect you. this can lead to something called septic shock,which can lead to death. maculopapular rash is another acute sign. macules are flat spots up to 1 cm, and papulesare bumps up to 1 cm. maculopapular is a combination between maculesand papules. photosensitivity can also be seen which meansthat the skin is much more sensitive to sunlight than normally.

this can cause cause solar urticaria, whichis a vascular reaction of the skin that cause pathches. patches are flat lesions like macules, butthey are bigger than 1 cm. so one difference between macules and patchesis the size. macules are less than 1 cm, whereas patchesare more than 1 cm. so the acute cutaneous lupus erythematosuscan be divided into 5 lesions: butterfly shaped erythema, bullous lesions, toxic epidermalnecrolysis, maculopapular rashes, and photosensitive solar urticarias. subacute cutaneous lupus erythematosus canbe divided into 2 types.

one is annular polycyclic lesions. the word polycyclic refers to more than 1ring structures that can be seen in chemistry for example. the word annular also refers to a ring-shapedstructure. so annular polycyclic lesions are ring-shapedskin lesions that usually occur on sun-exposed areas of the skin. nonindurated psoriaform is the other typeof subacute skin lesions. psoriaform means that it looks like psoriasis. nonindurated means that it is not hardas psoriasis.

subacute can be divided into annular polycyclicand nonindurated psoriaform lesions chronic skin lesions are chilblain lupus,that affects toes, fingers, nose, and ears during cold weather. these are painful, bright red nodules. nodules means a swelling of the skin thatis up to 1 cm diameter. discoid rash can also be seen. discoid refers to the disc shape of theselesions. we can divide discoid into classical type. discoid lesions can be divided into localized,which means that discoid lesions appear above

the neck,and generalized, which means that discoid lesions can appear both above and below theneck. lichen planus is an inflammatory disease thatcan affect the skin and oral mucosa. here we see a picture of whitish lichen planuson the oral mucosa. the word lichen means tree-moss as you cansee here on the picture. so this disease is looking like a tree mossbut with a whitish color. in sle patients this happen, that a discoidlesion is seen together with lichen planus. verrucous lesions or also called hypertrophiclesions are skin that hypertrophies, which means that the skin cells increase in numberand cause these hard wartlike lesions, especially

on extensor arms. mucosal lesions can affect the mucosal membranearound the teeth, the tongue, and hardpalate panniculitis or also called profundus, isan inflammation and destruction of the subcutaneous fat. tumidus lesions are pinkish urticarial non-scarringplaques usually in sun-exposed areas. plaques are elevation of the skin similarto papule, but they differ in size. so plaques are more than 1 cm in diameter,whereas papules are less than 1 cm. the chronic skin lesion are chilblain, discoid,discoid with lichen planus, hypertrophic, mucosal, panniculitis, and tumidus.

ulcers can appear on the mucosal membrane,on for example the hardpalate, buccal region, on the tongue, and on the nasal septum. inflammation can affect the heart causingpericarditis. here we see a normal normal heart on the upperpicture, and a reddish heart on the lower picture. inflammation is causing redness, pain, heat,swelling and loss of function. the heart wall rub against each other causinga typical pericardial rub sound that one can hear with a stethoscope. here we see a man having a painful pericarditispericardial effusion can also happen, which

means that fluid accumulates around the heartcausing a typical waterbottle-shaped heart on x-ray. as i mentioned, pericardial rub sounds canbe heard with a stethoscope. inflammation can also affect the lungs. so similar to the heart, there will be inflammation,pain, pleural friction rub sounds, and pleural effusionhere we can see the pleural effusion that have accumulated between the 2 layers of thelungs. this is how pleural friction rub sounds like. imagine walking on snow when you hear thissound.

we need to collect urine to find kidney problems. more than 500 mg of protein in the urine duringa 24 hour period can be seen in sle patients. this is called proteinuria, meaning proteinin the urine, which is not normal. we can also find red blood cell casts in theurine by analyzing the urine in a microscope. these casts are formed by red blood cellsthat stick together. blood in the urine is not normal. it suggest that something is wrong with thekidney. synovitis is inflammation of the synovialmembrane of the joints. polyarthritis can be seen in sle.

poly stands for that more than 1 joint isaffected. arthritis is inflammation of joint. so more than 1 joint will be inflamed causingpain. the nervous system can also be affected bysle. seizures can happen, which are hyperexcitationof neurons in the brain, sometimes leading to muscle contractions. psychosis and acute confusional state, alsocalled delirium can happen. psychosis and delirium patients usually sufferfrom hallucinations. neuropathies can be seen in sle.

neuropathy means neurons that are diseased. the neurons can be in the cranial part, peripheralpart, in the spinal cord which is then called myelitis, or single nerves can be damagedwhich is then called mononeuropathy, mono standing for single. or multiple single nerves in different areasof the body can be damaged, which is then called multiple mononeuropathy. what can we see in the blood? with a microscope we can distinguish differentblood cells. here is a picture showing the blood cells.

the blood cells originate from one cell calledmultipotential hematopoetic stem cell. this cell can produce myeloid cells, and lymphoidcells. the myeloid cells can produce erythrocytes,also called red blood cells. but in sle hemolytic anemia can be seen, whichmeans that the erythrocytes can damaged and therefore anemia will happen, which meansthat not enough oxygen is transported to the tissues. the myeloid cells can also produce megakaryocytes,that can further produce thrombocytes, also called platelets. but in sle the number of thrombocytes canbe reduced, and we call this thrombocytopenia.

penia stands for less of something, inthis case we have a penia of thrombocytes, so thrombocytopenia. the symptom of thrombocytopenia is usuallypetechiae which are small purplish spots on the skin. leukopenia can happen in sle which is a reducednumber of leukocytes, which are white blood cells, with neutrophils being the most commonwhite blood cell. lymphopenia can also be seen, which are reducednumber of lymphocytes. so the clinical criteria were alopecia, skinlesions, ulcers, diseases of the heart, lungs, kidneys, joints, nervous system and the blood.

the immunologic criteria are related to autoantibodies,meaning that the antibodies of the immune system attacks itself, instead of only attackingbacterias etc. here we have a cell, with its nucleus, containingchromosomes that contain dna. in sle, the antibodies attack your own nuclearproteins, for example your own dna, or rna binding proteins called smith proteins. so in sle we check for antinuclear antibodylevels, abbreviated as ana. we also check anti-smith and anti-double strandeddna. another immunologic criteria is direct coombstest. here we mix the patients blood together witha coombs reagent, and if red blood cells agglutinate,

meaning they stick together, then we havea positive test result. the complement system helps or complementsthe antibodies to fight infections. in sle we can see a low number of these complementproteins. the complement proteins are numbered c1 toc9, and it is the c3 and c4 complement proteins that are low in sle. ch50 stands for the total complement activity,which is also low in sle. an increased number of antiphospholipid antibodiescan be measured in sle. these antibodies bind to proteins like beta2 glycoprotein 1 on the phospholipid cell membrane.

the function of the beta 2 glycoprotein 1is to prevent phospholipid membrane to activate the thrombosis cascade. we know that thrombosis can cause death. so therefore it is very important to checkthe antiphospolipid antibody level. it is especially important in pregnant women,because these antibodies can cause spontaneous abortion and late fetal death. except antiphospholipid antibodies, sle flarescan also cause fetal death. sle fluctuate between flares and remissions. a flare is a very active disease with manysymptoms, whereas a remission is an inactive

state with few symptoms. during pregnancy, the mother should be monitoredfor any sle flares since these can lead to fetal death. so if a woman wants a child, then pregnancyshould be timed for when sle is in remission for at least 6 months. furthermore, anti-ro antibodies should bemeasured, because if anti-ro is detected, then doctors should warn mothers that thereare risks of the fetus getting a neonatal lupus or even a severe congenital heart blockwhich means the death of the fetus. so it is very important to monitor the fetalheart in this case, with for example an echocardiograph

and a 24-hour holter monitor. lets turn now to the biopsy criteria. here we need a biopsy of the kidney, whichwill show that there is inflammation, called nephritis. but it is not enough with a biopsy, we needan immunologic criteria as well, for example antinuclear antibodies, or anti-double-strandeddna. now lets see how we can treat sle patients. the first thing that we have to do is to removeany type of medication that can cause sle-like symptoms.

these are for example hydralazine, procainamide,and isoniazid. non-steroidal anti-inflammatory drugs arevery useful in sle patients, especially in controling arthralgias, which means painfuljoints. here we can use naproxen, ibuprofen, and diclofenac. antimalarial medications also help jointsproblems, but also skin problems, and they reduces the sle flares. the typical sle medication is hydroxychloroquine,but there are alternative like chloroquine and quinacrine. hydroxychloroquine can in rare cases causeretinal problems, skeletal muscle problems

and cardiac problems. so it is important that the eyes are examinedyearly. corticosteroids are usually the first linetreatment in acute severe sle. we typically begin by giving intravenous methylprednisolonefor 3 days and then we maintain the therapy with prednisone. disease-modifying antirheumatic drugs arealso very important. we can use azathioprine, methotrexate, mycophenolatemofetil, cyclophosphamide together with mesna, and in very severe cases intravenous immunoglobulin. it is well known that corticosteroid use fora long time can cause osteoporosis, which

means bone weakening. therefore it is important to consider givingcalcium, vitamin d, and bisphosphonate. so to conclude, we can say that emma has sle,which is a systemic autoimmune disease, that affects her whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, and the vascular system. the typical butterfly-shaped erythema canbe seen in sle patients. thank you very much for listening!

what is panniculitis

systemic lupus erythematosus, abbreviatedas sle. this is a systemic disease, which means thatit can affect the whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, the vascular system, and the list goes on. lupus means wolf. erythematosus means reddening of the skin. why did i show a red butterfly? because a very typical sign of sle is a butterflyshaped reddening of the skin. the way i remember this disease, is to imaginea wolf that attacks a woman, and why a woman?

because 9 out 10 patients are women. so the wolf bites the face of the woman causinga butterfly shaped erythema on her face. then the wolf continues to bite all her organsone by one, because this disease is a systemic disease, which means that it can affect allthe organs systemically. of course this disease has nothing to do witha wolf. but sle is an autoimmune disease, which meansthat this woman called emma have an immune system that attacks her own body, insteadof only attacking bacterias, viruses and parasites. so emma have an immune system that acts asa wolf inside her.

i will show you how we can find out if emmareally has sle by diagnosing her. and then we will look at what type of medicationswe can give her. i want you to remember at least one thingfrom this presentation. that is systemic lupus international collaboratingclinics, abbreviated as slicc. slicc is a criterion that can help in thediagnosis of sle. slicc contains clinical criteria, immunologiccriteria and biopsy criteria. we need more than 4 criteria with at least1 clinical and 1 immunologic. or we need 1 biopsy criteria and 1 immunologiccriteria.

so what does these 3 words mean? clinical, immunologic and biopsy? the synonym for clinic, is hospital. so clinical criteria stand for the symptoms,signs and lab values of a patient in the hospital. immunology deals with the immune system thatprotects you from bacterias, viruses, and immunologic criteria are usually lab teststhat detect any problem with the immune system. biopsy means that a doctor cuts out a pieceof an organ. for example, a small piece of kidney can becut out, and then the doctor puts this piece of kidney in a microscope and looks for diseases.

clinical criteria can be divided into alopecia,which means hairloss. skin problemsulcers heart diseaseslung diseases kidney problemsjoint pain problems with the nervous systemand blood diseases. skin problems can be grouped under the namecutaneous lupus erythematosus. we can divide these skin diseases into acutesubacute and chronic, based on the timing of the diseases. acute diseases are usually those that happensuddenly with a short duration.

chronic diseases are ongoing for a long time. whereas subacute means those diseases thatare not yet chronic but has passed the acute phase. a way to remember these timing are to memorize2 numbers. 1 and 3. why? because the duration of acute diseases areusually less than 1 month, subacute are 1 to 3 months, and chronic are more than 3 monthsin duration. so which are the acute type of skin problems?

butterfly erythema, which is a reddening ofthe face in a butterfly shape. it usually affects the cheeks or also calledmalar region, and therefore it is sometimes called malar erythema. it does not affect the nasolabial region. there can also be bullous lesions which areblisters that can be a mix of small grouped vesicles to large tense bullae. bullae are elevating the skin and are filledwith fluid. toxic epidermal necrolysis-like sle, is adetachment of the epidermis, the upperlayer of the skin, resulting in exfoliation, meaningthat the upperlayer just falls off.

this is very dangerous, because the skin isthe organ that protects against infections. so if you loose a large part of your skin,then sepsis can happen, which means that bacterias easily invade your vascular system, sincethere is no skin to protect you. this can lead to something called septic shock,which can lead to death. maculopapular rash is another acute sign. macules are flat spots up to 1 cm, and papulesare bumps up to 1 cm. maculopapular is a combination between maculesand papules. photosensitivity can also be seen which meansthat the skin is much more sensitive to sunlight than normally.

this can cause cause solar urticaria, whichis a vascular reaction of the skin that cause pathches. patches are flat lesions like macules, butthey are bigger than 1 cm. so one difference between macules and patchesis the size. macules are less than 1 cm, whereas patchesare more than 1 cm. so the acute cutaneous lupus erythematosuscan be divided into 5 lesions: butterfly shaped erythema, bullous lesions, toxic epidermalnecrolysis, maculopapular rashes, and photosensitive solar urticarias. subacute cutaneous lupus erythematosus canbe divided into 2 types.

one is annular polycyclic lesions. the word polycyclic refers to more than 1ring structures that can be seen in chemistry for example. the word annular also refers to a ring-shapedstructure. so annular polycyclic lesions are ring-shapedskin lesions that usually occur on sun-exposed areas of the skin. nonindurated psoriaform is the other typeof subacute skin lesions. psoriaform means that it looks like psoriasis. nonindurated means that it is not hardas psoriasis.

subacute can be divided into annular polycyclicand nonindurated psoriaform lesions chronic skin lesions are chilblain lupus,that affects toes, fingers, nose, and ears during cold weather. these are painful, bright red nodules. nodules means a swelling of the skin thatis up to 1 cm diameter. discoid rash can also be seen. discoid refers to the disc shape of theselesions. we can divide discoid into classical type. discoid lesions can be divided into localized,which means that discoid lesions appear above

the neck,and generalized, which means that discoid lesions can appear both above and below theneck. lichen planus is an inflammatory disease thatcan affect the skin and oral mucosa. here we see a picture of whitish lichen planuson the oral mucosa. the word lichen means tree-moss as you cansee here on the picture. so this disease is looking like a tree mossbut with a whitish color. in sle patients this happen, that a discoidlesion is seen together with lichen planus. verrucous lesions or also called hypertrophiclesions are skin that hypertrophies, which means that the skin cells increase in numberand cause these hard wartlike lesions, especially

on extensor arms. mucosal lesions can affect the mucosal membranearound the teeth, the tongue, and hardpalate panniculitis or also called profundus, isan inflammation and destruction of the subcutaneous fat. tumidus lesions are pinkish urticarial non-scarringplaques usually in sun-exposed areas. plaques are elevation of the skin similarto papule, but they differ in size. so plaques are more than 1 cm in diameter,whereas papules are less than 1 cm. the chronic skin lesion are chilblain, discoid,discoid with lichen planus, hypertrophic, mucosal, panniculitis, and tumidus.

ulcers can appear on the mucosal membrane,on for example the hardpalate, buccal region, on the tongue, and on the nasal septum. inflammation can affect the heart causingpericarditis. here we see a normal normal heart on the upperpicture, and a reddish heart on the lower picture. inflammation is causing redness, pain, heat,swelling and loss of function. the heart wall rub against each other causinga typical pericardial rub sound that one can hear with a stethoscope. here we see a man having a painful pericarditispericardial effusion can also happen, which

means that fluid accumulates around the heartcausing a typical waterbottle-shaped heart on x-ray. as i mentioned, pericardial rub sounds canbe heard with a stethoscope. inflammation can also affect the lungs. so similar to the heart, there will be inflammation,pain, pleural friction rub sounds, and pleural effusionhere we can see the pleural effusion that have accumulated between the 2 layers of thelungs. this is how pleural friction rub sounds like. imagine walking on snow when you hear thissound.

we need to collect urine to find kidney problems. more than 500 mg of protein in the urine duringa 24 hour period can be seen in sle patients. this is called proteinuria, meaning proteinin the urine, which is not normal. we can also find red blood cell casts in theurine by analyzing the urine in a microscope. these casts are formed by red blood cellsthat stick together. blood in the urine is not normal. it suggest that something is wrong with thekidney. synovitis is inflammation of the synovialmembrane of the joints. polyarthritis can be seen in sle.

poly stands for that more than 1 joint isaffected. arthritis is inflammation of joint. so more than 1 joint will be inflamed causingpain. the nervous system can also be affected bysle. seizures can happen, which are hyperexcitationof neurons in the brain, sometimes leading to muscle contractions. psychosis and acute confusional state, alsocalled delirium can happen. psychosis and delirium patients usually sufferfrom hallucinations. neuropathies can be seen in sle.

neuropathy means neurons that are diseased. the neurons can be in the cranial part, peripheralpart, in the spinal cord which is then called myelitis, or single nerves can be damagedwhich is then called mononeuropathy, mono standing for single. or multiple single nerves in different areasof the body can be damaged, which is then called multiple mononeuropathy. what can we see in the blood? with a microscope we can distinguish differentblood cells. here is a picture showing the blood cells.

the blood cells originate from one cell calledmultipotential hematopoetic stem cell. this cell can produce myeloid cells, and lymphoidcells. the myeloid cells can produce erythrocytes,also called red blood cells. but in sle hemolytic anemia can be seen, whichmeans that the erythrocytes can damaged and therefore anemia will happen, which meansthat not enough oxygen is transported to the tissues. the myeloid cells can also produce megakaryocytes,that can further produce thrombocytes, also called platelets. but in sle the number of thrombocytes canbe reduced, and we call this thrombocytopenia.

penia stands for less of something, inthis case we have a penia of thrombocytes, so thrombocytopenia. the symptom of thrombocytopenia is usuallypetechiae which are small purplish spots on the skin. leukopenia can happen in sle which is a reducednumber of leukocytes, which are white blood cells, with neutrophils being the most commonwhite blood cell. lymphopenia can also be seen, which are reducednumber of lymphocytes. so the clinical criteria were alopecia, skinlesions, ulcers, diseases of the heart, lungs, kidneys, joints, nervous system and the blood.

the immunologic criteria are related to autoantibodies,meaning that the antibodies of the immune system attacks itself, instead of only attackingbacterias etc. here we have a cell, with its nucleus, containingchromosomes that contain dna. in sle, the antibodies attack your own nuclearproteins, for example your own dna, or rna binding proteins called smith proteins. so in sle we check for antinuclear antibodylevels, abbreviated as ana. we also check anti-smith and anti-double strandeddna. another immunologic criteria is direct coombstest. here we mix the patients blood together witha coombs reagent, and if red blood cells agglutinate,

meaning they stick together, then we havea positive test result. the complement system helps or complementsthe antibodies to fight infections. in sle we can see a low number of these complementproteins. the complement proteins are numbered c1 toc9, and it is the c3 and c4 complement proteins that are low in sle. ch50 stands for the total complement activity,which is also low in sle. an increased number of antiphospholipid antibodiescan be measured in sle. these antibodies bind to proteins like beta2 glycoprotein 1 on the phospholipid cell membrane.

the function of the beta 2 glycoprotein 1is to prevent phospholipid membrane to activate the thrombosis cascade. we know that thrombosis can cause death. so therefore it is very important to checkthe antiphospolipid antibody level. it is especially important in pregnant women,because these antibodies can cause spontaneous abortion and late fetal death. except antiphospholipid antibodies, sle flarescan also cause fetal death. sle fluctuate between flares and remissions. a flare is a very active disease with manysymptoms, whereas a remission is an inactive

state with few symptoms. during pregnancy, the mother should be monitoredfor any sle flares since these can lead to fetal death. so if a woman wants a child, then pregnancyshould be timed for when sle is in remission for at least 6 months. furthermore, anti-ro antibodies should bemeasured, because if anti-ro is detected, then doctors should warn mothers that thereare risks of the fetus getting a neonatal lupus or even a severe congenital heart blockwhich means the death of the fetus. so it is very important to monitor the fetalheart in this case, with for example an echocardiograph

and a 24-hour holter monitor. lets turn now to the biopsy criteria. here we need a biopsy of the kidney, whichwill show that there is inflammation, called nephritis. but it is not enough with a biopsy, we needan immunologic criteria as well, for example antinuclear antibodies, or anti-double-strandeddna. now lets see how we can treat sle patients. the first thing that we have to do is to removeany type of medication that can cause sle-like symptoms.

these are for example hydralazine, procainamide,and isoniazid. non-steroidal anti-inflammatory drugs arevery useful in sle patients, especially in controling arthralgias, which means painfuljoints. here we can use naproxen, ibuprofen, and diclofenac. antimalarial medications also help jointsproblems, but also skin problems, and they reduces the sle flares. the typical sle medication is hydroxychloroquine,but there are alternative like chloroquine and quinacrine. hydroxychloroquine can in rare cases causeretinal problems, skeletal muscle problems

and cardiac problems. so it is important that the eyes are examinedyearly. corticosteroids are usually the first linetreatment in acute severe sle. we typically begin by giving intravenous methylprednisolonefor 3 days and then we maintain the therapy with prednisone. disease-modifying antirheumatic drugs arealso very important. we can use azathioprine, methotrexate, mycophenolatemofetil, cyclophosphamide together with mesna, and in very severe cases intravenous immunoglobulin. it is well known that corticosteroid use fora long time can cause osteoporosis, which

means bone weakening. therefore it is important to consider givingcalcium, vitamin d, and bisphosphonate. so to conclude, we can say that emma has sle,which is a systemic autoimmune disease, that affects her whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, and the vascular system. the typical butterfly-shaped erythema canbe seen in sle patients. thank you very much for listening!

lupus profundus

systemic lupus erythematosus, abbreviatedas sle. this is a systemic disease, which means thatit can affect the whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, the vascular system, and the list goes on. lupus means wolf. erythematosus means reddening of the skin. why did i show a red butterfly? because a very typical sign of sle is a butterflyshaped reddening of the skin. the way i remember this disease, is to imaginea wolf that attacks a woman, and why a woman?

because 9 out 10 patients are women. so the wolf bites the face of the woman causinga butterfly shaped erythema on her face. then the wolf continues to bite all her organsone by one, because this disease is a systemic disease, which means that it can affect allthe organs systemically. of course this disease has nothing to do witha wolf. but sle is an autoimmune disease, which meansthat this woman called emma have an immune system that attacks her own body, insteadof only attacking bacterias, viruses and parasites. so emma have an immune system that acts asa wolf inside her.

i will show you how we can find out if emmareally has sle by diagnosing her. and then we will look at what type of medicationswe can give her. i want you to remember at least one thingfrom this presentation. that is systemic lupus international collaboratingclinics, abbreviated as slicc. slicc is a criterion that can help in thediagnosis of sle. slicc contains clinical criteria, immunologiccriteria and biopsy criteria. we need more than 4 criteria with at least1 clinical and 1 immunologic. or we need 1 biopsy criteria and 1 immunologiccriteria.

so what does these 3 words mean? clinical, immunologic and biopsy? the synonym for clinic, is hospital. so clinical criteria stand for the symptoms,signs and lab values of a patient in the hospital. immunology deals with the immune system thatprotects you from bacterias, viruses, and immunologic criteria are usually lab teststhat detect any problem with the immune system. biopsy means that a doctor cuts out a pieceof an organ. for example, a small piece of kidney can becut out, and then the doctor puts this piece of kidney in a microscope and looks for diseases.

clinical criteria can be divided into alopecia,which means hairloss. skin problemsulcers heart diseaseslung diseases kidney problemsjoint pain problems with the nervous systemand blood diseases. skin problems can be grouped under the namecutaneous lupus erythematosus. we can divide these skin diseases into acutesubacute and chronic, based on the timing of the diseases. acute diseases are usually those that happensuddenly with a short duration.

chronic diseases are ongoing for a long time. whereas subacute means those diseases thatare not yet chronic but has passed the acute phase. a way to remember these timing are to memorize2 numbers. 1 and 3. why? because the duration of acute diseases areusually less than 1 month, subacute are 1 to 3 months, and chronic are more than 3 monthsin duration. so which are the acute type of skin problems?

butterfly erythema, which is a reddening ofthe face in a butterfly shape. it usually affects the cheeks or also calledmalar region, and therefore it is sometimes called malar erythema. it does not affect the nasolabial region. there can also be bullous lesions which areblisters that can be a mix of small grouped vesicles to large tense bullae. bullae are elevating the skin and are filledwith fluid. toxic epidermal necrolysis-like sle, is adetachment of the epidermis, the upperlayer of the skin, resulting in exfoliation, meaningthat the upperlayer just falls off.

this is very dangerous, because the skin isthe organ that protects against infections. so if you loose a large part of your skin,then sepsis can happen, which means that bacterias easily invade your vascular system, sincethere is no skin to protect you. this can lead to something called septic shock,which can lead to death. maculopapular rash is another acute sign. macules are flat spots up to 1 cm, and papulesare bumps up to 1 cm. maculopapular is a combination between maculesand papules. photosensitivity can also be seen which meansthat the skin is much more sensitive to sunlight than normally.

this can cause cause solar urticaria, whichis a vascular reaction of the skin that cause pathches. patches are flat lesions like macules, butthey are bigger than 1 cm. so one difference between macules and patchesis the size. macules are less than 1 cm, whereas patchesare more than 1 cm. so the acute cutaneous lupus erythematosuscan be divided into 5 lesions: butterfly shaped erythema, bullous lesions, toxic epidermalnecrolysis, maculopapular rashes, and photosensitive solar urticarias. subacute cutaneous lupus erythematosus canbe divided into 2 types.

one is annular polycyclic lesions. the word polycyclic refers to more than 1ring structures that can be seen in chemistry for example. the word annular also refers to a ring-shapedstructure. so annular polycyclic lesions are ring-shapedskin lesions that usually occur on sun-exposed areas of the skin. nonindurated psoriaform is the other typeof subacute skin lesions. psoriaform means that it looks like psoriasis. nonindurated means that it is not hardas psoriasis.

subacute can be divided into annular polycyclicand nonindurated psoriaform lesions chronic skin lesions are chilblain lupus,that affects toes, fingers, nose, and ears during cold weather. these are painful, bright red nodules. nodules means a swelling of the skin thatis up to 1 cm diameter. discoid rash can also be seen. discoid refers to the disc shape of theselesions. we can divide discoid into classical type. discoid lesions can be divided into localized,which means that discoid lesions appear above

the neck,and generalized, which means that discoid lesions can appear both above and below theneck. lichen planus is an inflammatory disease thatcan affect the skin and oral mucosa. here we see a picture of whitish lichen planuson the oral mucosa. the word lichen means tree-moss as you cansee here on the picture. so this disease is looking like a tree mossbut with a whitish color. in sle patients this happen, that a discoidlesion is seen together with lichen planus. verrucous lesions or also called hypertrophiclesions are skin that hypertrophies, which means that the skin cells increase in numberand cause these hard wartlike lesions, especially

on extensor arms. mucosal lesions can affect the mucosal membranearound the teeth, the tongue, and hardpalate panniculitis or also called profundus, isan inflammation and destruction of the subcutaneous fat. tumidus lesions are pinkish urticarial non-scarringplaques usually in sun-exposed areas. plaques are elevation of the skin similarto papule, but they differ in size. so plaques are more than 1 cm in diameter,whereas papules are less than 1 cm. the chronic skin lesion are chilblain, discoid,discoid with lichen planus, hypertrophic, mucosal, panniculitis, and tumidus.

ulcers can appear on the mucosal membrane,on for example the hardpalate, buccal region, on the tongue, and on the nasal septum. inflammation can affect the heart causingpericarditis. here we see a normal normal heart on the upperpicture, and a reddish heart on the lower picture. inflammation is causing redness, pain, heat,swelling and loss of function. the heart wall rub against each other causinga typical pericardial rub sound that one can hear with a stethoscope. here we see a man having a painful pericarditispericardial effusion can also happen, which

means that fluid accumulates around the heartcausing a typical waterbottle-shaped heart on x-ray. as i mentioned, pericardial rub sounds canbe heard with a stethoscope. inflammation can also affect the lungs. so similar to the heart, there will be inflammation,pain, pleural friction rub sounds, and pleural effusionhere we can see the pleural effusion that have accumulated between the 2 layers of thelungs. this is how pleural friction rub sounds like. imagine walking on snow when you hear thissound.

we need to collect urine to find kidney problems. more than 500 mg of protein in the urine duringa 24 hour period can be seen in sle patients. this is called proteinuria, meaning proteinin the urine, which is not normal. we can also find red blood cell casts in theurine by analyzing the urine in a microscope. these casts are formed by red blood cellsthat stick together. blood in the urine is not normal. it suggest that something is wrong with thekidney. synovitis is inflammation of the synovialmembrane of the joints. polyarthritis can be seen in sle.

poly stands for that more than 1 joint isaffected. arthritis is inflammation of joint. so more than 1 joint will be inflamed causingpain. the nervous system can also be affected bysle. seizures can happen, which are hyperexcitationof neurons in the brain, sometimes leading to muscle contractions. psychosis and acute confusional state, alsocalled delirium can happen. psychosis and delirium patients usually sufferfrom hallucinations. neuropathies can be seen in sle.

neuropathy means neurons that are diseased. the neurons can be in the cranial part, peripheralpart, in the spinal cord which is then called myelitis, or single nerves can be damagedwhich is then called mononeuropathy, mono standing for single. or multiple single nerves in different areasof the body can be damaged, which is then called multiple mononeuropathy. what can we see in the blood? with a microscope we can distinguish differentblood cells. here is a picture showing the blood cells.

the blood cells originate from one cell calledmultipotential hematopoetic stem cell. this cell can produce myeloid cells, and lymphoidcells. the myeloid cells can produce erythrocytes,also called red blood cells. but in sle hemolytic anemia can be seen, whichmeans that the erythrocytes can damaged and therefore anemia will happen, which meansthat not enough oxygen is transported to the tissues. the myeloid cells can also produce megakaryocytes,that can further produce thrombocytes, also called platelets. but in sle the number of thrombocytes canbe reduced, and we call this thrombocytopenia.

penia stands for less of something, inthis case we have a penia of thrombocytes, so thrombocytopenia. the symptom of thrombocytopenia is usuallypetechiae which are small purplish spots on the skin. leukopenia can happen in sle which is a reducednumber of leukocytes, which are white blood cells, with neutrophils being the most commonwhite blood cell. lymphopenia can also be seen, which are reducednumber of lymphocytes. so the clinical criteria were alopecia, skinlesions, ulcers, diseases of the heart, lungs, kidneys, joints, nervous system and the blood.

the immunologic criteria are related to autoantibodies,meaning that the antibodies of the immune system attacks itself, instead of only attackingbacterias etc. here we have a cell, with its nucleus, containingchromosomes that contain dna. in sle, the antibodies attack your own nuclearproteins, for example your own dna, or rna binding proteins called smith proteins. so in sle we check for antinuclear antibodylevels, abbreviated as ana. we also check anti-smith and anti-double strandeddna. another immunologic criteria is direct coombstest. here we mix the patients blood together witha coombs reagent, and if red blood cells agglutinate,

meaning they stick together, then we havea positive test result. the complement system helps or complementsthe antibodies to fight infections. in sle we can see a low number of these complementproteins. the complement proteins are numbered c1 toc9, and it is the c3 and c4 complement proteins that are low in sle. ch50 stands for the total complement activity,which is also low in sle. an increased number of antiphospholipid antibodiescan be measured in sle. these antibodies bind to proteins like beta2 glycoprotein 1 on the phospholipid cell membrane.

the function of the beta 2 glycoprotein 1is to prevent phospholipid membrane to activate the thrombosis cascade. we know that thrombosis can cause death. so therefore it is very important to checkthe antiphospolipid antibody level. it is especially important in pregnant women,because these antibodies can cause spontaneous abortion and late fetal death. except antiphospholipid antibodies, sle flarescan also cause fetal death. sle fluctuate between flares and remissions. a flare is a very active disease with manysymptoms, whereas a remission is an inactive

state with few symptoms. during pregnancy, the mother should be monitoredfor any sle flares since these can lead to fetal death. so if a woman wants a child, then pregnancyshould be timed for when sle is in remission for at least 6 months. furthermore, anti-ro antibodies should bemeasured, because if anti-ro is detected, then doctors should warn mothers that thereare risks of the fetus getting a neonatal lupus or even a severe congenital heart blockwhich means the death of the fetus. so it is very important to monitor the fetalheart in this case, with for example an echocardiograph

and a 24-hour holter monitor. lets turn now to the biopsy criteria. here we need a biopsy of the kidney, whichwill show that there is inflammation, called nephritis. but it is not enough with a biopsy, we needan immunologic criteria as well, for example antinuclear antibodies, or anti-double-strandeddna. now lets see how we can treat sle patients. the first thing that we have to do is to removeany type of medication that can cause sle-like symptoms.

these are for example hydralazine, procainamide,and isoniazid. non-steroidal anti-inflammatory drugs arevery useful in sle patients, especially in controling arthralgias, which means painfuljoints. here we can use naproxen, ibuprofen, and diclofenac. antimalarial medications also help jointsproblems, but also skin problems, and they reduces the sle flares. the typical sle medication is hydroxychloroquine,but there are alternative like chloroquine and quinacrine. hydroxychloroquine can in rare cases causeretinal problems, skeletal muscle problems

and cardiac problems. so it is important that the eyes are examinedyearly. corticosteroids are usually the first linetreatment in acute severe sle. we typically begin by giving intravenous methylprednisolonefor 3 days and then we maintain the therapy with prednisone. disease-modifying antirheumatic drugs arealso very important. we can use azathioprine, methotrexate, mycophenolatemofetil, cyclophosphamide together with mesna, and in very severe cases intravenous immunoglobulin. it is well known that corticosteroid use fora long time can cause osteoporosis, which

means bone weakening. therefore it is important to consider givingcalcium, vitamin d, and bisphosphonate. so to conclude, we can say that emma has sle,which is a systemic autoimmune disease, that affects her whole body systemically. the brain, skin, heart, lungs, kidneys, joints,the immune system, and the vascular system. the typical butterfly-shaped erythema canbe seen in sle patients. thank you very much for listening!

abdominal panniculitis

- [voiceover] so herewe have our vascular man and he's got blood vessels that supply every part of the body. he's got blood vesselssupplying the heart, blood vessels supplying the lungs, some supplying the kidney, the liver, the intestines, the skin, the nerves, really all over the place. so here's a blood vessel i'm drawing,

of course your blood vessels will be carrying blood, but they also carry nutrients and oxygen and all sorts of proteins. now what happens if theseblood vessels get damaged, or inflamed? what if the inside of the wall of the blood vessel gets very inflamed? well intuitively it makes sense,

blood will not be able topass as well through here and be delivered to the different organs. you know the intestines,the livers, the lung. all of the organs of the body need blood and need nutrients. this damage is precisely what happens in the disease known as vasculitis. vasculitis is damage of blood vessels and inflammation of blood vessels.

itis means inflammation and vascul means vasculatureor blood vessels. essentially this damage iscaused by the immune system. white blood cells mistakenlyrelease small molecules that can damage the blood vessels. essentially the immunesystem makes a mistake and thinks that blood vessels are foreign. so vasculitis is an autoimmune disease. now i know what you're probably thinking,

you might be thinking if i destroy all my blood vessels, how is that compatible with life? well there are differenttypes of vasculitides, the plural for vasculitis and these different typesmight affect different parts of the body. for example one type ofvasculitis might affect the lungs and the kidneys only.

another type might affectthe intestines, the kidney, the heart and the lungs and still another type might only affect the big blood vessels thatcome out of the heart. the different organs affected in patients lead to the differentsymptoms that you might see. for example loss ofblood flow and nutrients to the heart tissuemeans heart cell death, this is known as a heart attack

and this might causesymptoms such as chest pain. the severity of symptomsmight also be different, so for example with abdominal pain a patient might have a range from a small amount of blood in their stool to full on bowel perforation. this all depends on how severely the blood vessels are damaged. now along with these local symptoms

patients might alsoexperience general symptoms such as night sweats or fever, so there's a littlethermometer right here, as the patient might have a fever or the patient might have chills or generalized muscle aches, or they may also experience lethargy or a feeling of being very tired. this all comes from what'scausing this problem,

remember white blood cells are releasing little immune molecules, these immune moleculescan travel down to the rest of the body. these immune molecules are normally used to fight off pathogens and so a patient might feel like they have a general illness or a virus. now let's take a step back.

why are only certaintypes of vessels affected in vasculitis? the different types ofvessels that are affected usually depends on thesize of those blood vessels and so vasculitis has been classified into three different categories. large vessel vasculitis, medium vessel vasculitis and small vessel vasculitis.

here i'll draw a blood vessel to show a little bitabout what's going on. here let me draw this large blood vessel and i've got the blood vessel wall and the outside and theinside of the blood vessel, out and in, and of course on theinside you have the things i have mentioned before. blood, oxygen, nutrients, that all travel

through your blood vesselslike water through pipes. now the purpose of large blood vessels is to get blood distributed quickly through the body to where it needs to go. so if we have inflammation and damage of the blood vessel wall so it's bulging out frominflammation, swelling, scaring and then repeating that process, the blood trying to passthrough can't do so effectively

and so there's decreased blood flow and also after this constrictionyou'll see decreased blood pressure as well. and now a physicianlistening over the skin using a stethoscope mayactually hear this blockage, it's the same thing that happens when you put pressure on a hose. if you put a kink in the hose, not only will water stopflowing through as quickly

but also if you listen at the kink you can hear that bloodtrying to rush through and that's the same thingthe physician hears. this is known as a bruit and if the physician feels the area they may also feel what's called thrill, this feeling of blood rushing through. now for medium sized blood vessels . when scaring occurs for these vessels

it can potentiallyblock flow all together. this leads to blood cells kind of getting stuck behind the blockageand little proteins in the blood known as clotting proteins can form a clot andcompletely stop blood flow. along with clot formation, you can also see the bloodvessel wall bulge out. this is due to increased pressure, the blood has nowhereto go so it pushes up

against the walls. and since medium sized bloodvessel walls are thinner they are prone to this bulging. it's kind of like whenyou take a water balloon and squeeze it on one area, all the water bulges toone side of the wall. the bulging and weakeningof the blood vessel walls are known as aneurysms. the most fear complicationfrom aneurysms is rupture,

leading to blood spillingout of the blood vessels. last of all the finalclassification of blood vessels are small blood vessels. and by small i meanmicroscopic so we've got blood cells marchingthrough nearly single file and a very thin blood vessel wall. you can imagine that damage to this wall can lead to breakage of theblood vessel really easily and depending on where the blood vessel is

that's where you might see symptoms. for example if the small bloodvessels are in the intestines you might see bloody stool. if the blood vessels are in the kidneys you might see bloody urine. if the blood vesselsare just under the skin you might actually see a rash that kind of gives a dotted patternwhere all these different small little blood vessels have ruptured.

so in general the symptomsyou see in vaculitis depends on where the blood vessels are that are affected, whatsize they are and how sever the damage is.