engleski

RECURRENT ANGIOEDEMA IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS

ntroduction: Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease that results from the interaction of multiple enviromental, immunological and genetic factors, causing inflammation and eventually damage in multiple organs. Its prevalence ranges from approximately 40 cases per 100, 000 and 15– 20% of cases present in children under 16 years of age. It has been suggested that peadiatric lupus patients have more aggressive disease course and an increased rate of more unusual initial clinical presentations compared with their adult counterparts. Clinical feature may include urticarial vasculitis with prevalence of up to 2, 2%, while angioedema is very rare. Objectives: We present a 15 year old female patient with SLE, urticarial vasculitis and angioedema. Methods: The disease started with nonspecific symptoms of malaise and headache and the patient was even put on klonazepam because of irregular electroencephalogram (EEG). She also developed autoimmune thrombocytopaenia and prednisone therapy was included for several months. Few months prior to admittance to our Department because of recurrent urticarial rash and angioedema allergy tests were performed and all were negative. During an episode of urticarial rash and vascullitis, skin biopsy was performed and leukocytoclastic vasculitis was diagnosed. Direct immnofolurescency showed IgA, IgG, IgM, C3 and C1q depostis. With symptoms that included malaise, fever, poliarthralgias, headache that developed, laboratory tests and skin biopsy findings SLE was suspected. Results: Antinuclear antibodies (ANA) titer was positive up to 1: 1280 (anti Ro 3+, anti Ro- 52 3+ i anti La 1+) and with low concentration of C3 and C4 ACR- criteria were met for SLE. Patient was treated with "pulse“ doses of metilprednisolone followed by maintenance doses. Nevertheless, she developed angioedema of lips, tounge nad eyelids with obstructive breathing. She recived adrenaline, glucocorticoides and antihistamines with poor effect and only after infusion of fresh frozen plasma, symptoms decreased. At the time, levels of C1 esterase inhibitor (C1-INH) were normal, while C1-INH function and C1q levels are still expected Conclusion: Angioedema is an unusual occurence in SLE, especially in children and it's mechanisms are heterogenous . SLE can be associated with hereditary and acquired deficinecies or dysfunction of C1-INH which leads to hyperactivation of complement classic pathway, bradykinin synthesis and incrased vascular permeability. Anti C1-INH autoantibodies seem to be uncommon in acquired angioedema (AAE) in SLE. In our patient, angioedema is thought to be most likely of the acquired form secondary to SLE since in hereditary angioedema (HAE) there is usually a family history of angioedema and earlier onset of symptoms. Although angioedema is generally self- limited disease, it may be life threatening causing the airway obstruction with mortality rate 15-33%.

SLE; ANGIOEDEMA; VASCULITIS

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