Naslov
Anti-neutrophil Cytoplasmic Antibody Positivity in Five Children with Systemic Lupus Erythemathosus-What is the Importance of this Finding?

Autori
Bobek, Dubravka ; Vuković, Jurica ; Malenica, Branko ; Rukavina, Iva ; Bojanić, Katarina ; Jelušić, Marija

Izvornik
Acta Dermatovenerologica Croatica (1330-027X) 22 (2014), 4; 264-270

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
SLE ; ANCA vasculitis ; childhood

Sažetak
Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterised by formation of antibodies against nuclear antigens. Vasculitis may be found in the course of SLE but rarely corresponds to an anti-neutrophil cytoplasmic antibodies (ANCA) - associated vasculitis (AAV. We report five cases of severe JSLE associated with AAV diagnosed between 1991 to 2011 in three University- based tertiary care centers. All patients (3 girls and 2 boys, aged 12 to 17) presented with severe clinical picture and following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis stage V (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2) and gastointestinal bleeding (n=1). All patients were proteinase 3 (PR3) ANCA positive, while one of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. Patients were treated with corticosteroids and immunossupresive drugs (cyclophosphamide and azathioprine). Remission of disease was achieved at three patients. Course of disease had been worsening in two patients and we started with Rituximab (anti- CD20). In conclusion, all of our patients presented as the most severe SLE patients that need to be diagnosed as soon as possible and treat very intensively. Since an coexistance of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment and course of diseases are experiences that can be of great help.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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