title: A therapeutic challenge: catastrophic anti-phospholipid syndrome with diffuse alveolar haemorrhage.
abstract: Diffuse alveolar haemorrhage (DAH) complicating primary catastrophic anti-phospholipid syndrome (CAPS) was diagnosed in a 50-year-old female patient. Treatment strategies are limited for this often life-threatening autoimmune disease that requires aggressive immunosuppression. In the absence of clinically validated treatment strategies, high-dose steroids associated with plasma exchange and eventually intravenous immunoglobulins were used to manage the disease. Its severity prompted the initiation of rituximab that was administered weekly for four consecutive weeks. Anticoagulation therapy, on the other hand, needed to be discontinued due to the major haemorrhagic episodes. This combination treatment provided an effective control of the CAPS-associated DAH and helped achieve clinical remission.
authors.affiliation: Service de Réanimation Médicale, Centre Hospitalier Universitaire de Nice, L'Archet, 151 Route de Saint-Antoine-Ginestière, 06200, Nice, France, martis.n@chu-nice.fr.
authors.forename: Nihal
authors.initials: N
authors.lastname: Martis
authors.forename: Eléa
authors.initials: E
authors.lastname: Blanchouin
authors.forename: Rémi
authors.initials: R
authors.lastname: Lazdunski
authors.forename: Sarah
authors.initials: S
authors.lastname: Lechtman
authors.forename: Alexandre
authors.initials: A
authors.lastname: Robert
authors.forename: Hervé
authors.initials: H
authors.lastname: Hyvernat
authors.forename: Denis
authors.initials: D
authors.lastname: Doyen
authors.forename: Jean
authors.initials: J
authors.lastname: Dellamonica
authors.forename: Gilles
authors.initials: G
authors.lastname: Bernardin
mesh_terms: D000925:Anticoagulants / Q000008:administration & dosage; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006470:Hemorrhage / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D008875:Middle Aged; D010951:Plasma Exchange; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous