Recurrent episodes of hemorrhagic alveolitis in relapsing catastrophic antiphospholipid syndrome: the same side of the dark moon

Catastrophic antiphospholipid syndrome (CAPS) is a rare variant of antiphospholipid syndrome characterized by widespread thrombotic microangiopathy and multiorgan failure. Clinically, CAPS signs and symptoms can mimic vasculitis of systemic lupus erythematosus, disseminated intravascular coagulation, and thrombotic thrombocytopenic purpura. CAPS is burdened by high mortality, nearly 50 % in most series. However, patients surviving the acute phase rarely suffer of CAPS relapses. Moreover, concomitant pulmonary hemorrhagic alveolitis is a very rare complication warranting an ominous prognosis. Only few reports of relapsing CAPS are described in literature, and pathogenetic mechanisms are poorly understood and the optimal treatment is yet unknown. We report a case of a young man suffering from multiple relapses of CAPS and recurrent hemorrhagic pulmonary alveolitis refractory to aggressive combination treatment.