Catastrophic antiphospholipid syndrome in a young girl with lupus

Summary 

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disorder, still of unknown cause, that can affect the respiratory system with variable manifestations, course and prognosis. Catastrophic antiphospholipid syndrome is an uncommon feature in SLE and is often fatal. We report a case of fatal systemic lupus erythematosus in a 17-year-old girl with a catastrophic antiphospholipid syndrome who presented a pulmonary embolism and septic shock due to a urinary tract infection. We discuss the nosology, clinical features, immune disorders, treatment and prognosis of the disease.

Keywords: Catastrophic antiphospholipid syndrome, Fatal lupus, Septic shock, Systemic lupus erythematosus, Urinary tract infection