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!
