title: Recurrent episodes of hemorrhagic alveolitis in relapsing catastrophic antiphospholipid syndrome: the same side of the dark moon.
abstract: 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.
authors.affiliation: Internal and Emergency Medicine Unit, Careggi University Hospital, Largo Brambilla 3, 50134, Florence, Italy.
authors.forename: Filippo
authors.initials: F
authors.lastname: Pieralli
authors.forename: Maddalena
authors.initials: M
authors.lastname: Grazzini
authors.forename: Vieri
authors.initials: V
authors.lastname: Vannucchi
authors.forename: Antonio
authors.initials: A
authors.lastname: Mancini
authors.forename: Daniele
authors.initials: D
authors.lastname: Cammelli
authors.forename: Carlo
authors.initials: C
authors.lastname: Nozzoli
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications*; D017809:Fatal Outcome; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008171:Lung Diseases / Q000209:etiology*; D008297:Male; D012008:Recurrence