Primary antiphospholipid syndrome presenting as antiphospholipid syndrome nephropathy: a case report.

Introduction: Primary antiphospholipid syndrome can be a difficult diagnosis in the absence of typical clinical features. We describe an unusual presentation of primary antiphospholipid syndrome mimicking vasculitis for which the only diagnostic clue on initial presentation was antiphospholipid syndrome nephropathy.

Case Presentation: A 29-year-old Sri Lankan woman presented with features mimicking vasculitis with no obvious clinical features of antiphospholipid syndrome. Classical symptoms of antiphospholipid syndrome only appeared months later. A retrospective analysis showed that the only evidence of antiphospholipid syndrome at her first presentation was antiphospholipid syndrome nephropathy on her renal biopsy.

Conclusions: A high degree of suspicion of antiphospholipid syndrome is needed when patients present with non-specific vasculitis features. It has a broad clinical impact as antiphospholipid syndrome can present to any clinician with rare manifestations such as nephropathy. This significantly adds to the advancement of knowledge as antiphospholipid syndrome nephropathy should be recognized as a true entity and considered as a classification criteria for antiphospholipid syndrome.