Sjögren sendromu ve şiddetli cilt tutulumu mevcut olan katastrofik antifosfolipid sendromu

Katastrofik antifosfolipid sendromu; nadir görülen hızlı seyirli mortalitesi yüksek antifosfolipid antikor sendromunun ciddi bir formudur. Sendrom, yaygın mikro trombozlara bağlı olarak multiorgan yetmezliğine neden olur. Bu olguda; 58 yaşında kadın hasta da alt extremite distal bölgeden başlayan ekimozlar üst bacak bölgesine ve üst extremiteye yayılan lezyonlar hızlı seyir göstererek nekrotik alanlara dönüştü. Histopatolojik incelemede dermiste intravasküler trombüsler tespit edilen hastanın laboratuvar bulgularında IgM anti-kardiyolipin antikor ve Lupus antikoagülan’ı pozitifti. Klinik seyir çok hızlı ilerleyen hastada akciğer ve böbrek tutulumu da eşlik etti. Mevcut bulgularla hastaya katastrofik antifosfolipid sendromu tanısı konuldu. Katastrofik antifosfolipid sendromu vakalarının üçte ikisin de, en sık enfeksiyonların tetiklemesine sekonder olarak gelişmektedir. Katastrofik antifosfolipid sendromu’na otoimmün hastalıklar özellikle sistemik lupus eritematozus eşlik etmektedir. Olgumuz; Sjögren sendromunun katastrofik APS etyolojisinde düşünülmesini gerektiğini vurgulamak amacıyla sunulmaktadır.

Sjögren’s syndrome and catastrophic antiphospholipid syndrome with severe skin involvement

Catastrophic antiphospholipid syndrome is a rare but rapidly progressing form of antiphospholipid antibody syndrome with high mortality. The syndrome causes multiorgan failure associated with diffuse micro-thromboses. Necrotic-appearing ecchymotic lesions emerging from the distal aspect of both lower extremities and progressing to the upper leg region, and to the upper extremities developed in a 58-year-old woman. Histopathological examination of the biopsy specimen revealed intravascular microthrombi in the dermis. Laboratory findings for anti-cardiolipin antibodies IgM and lupus anticoagulant resulted positive. Lung and kidney involvement was observed. The clinical course progressed very quickly, and catastrophic antiphospholipid syndrome was diagnosed with these findings. Two-thirds of catastrophic antiphospholipid syndrome cases develop due to secondary causes, the most common being infections. Catastrophic antiphospholipid syndrome may also accompany autoimmune diseases, particularly systemic lupus erythematosus. This report is presented to emphasize that Sjögren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.

Keywords:

Antiphospholipid syndrome, catastrophic, sjögren syndrome,

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