Ralph Yachoui1, Rahul Sehgal2, Barkha Amlani3, Jerry W Goldberg2. 1. Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449. Electronic address: yachoui.ralph@marshfieldclinic.org. 2. Department of Rheumatology, Marshfield Clinic, Marshfield, 1000 N Oak Ave, WI 54449. 3. Department of Internal Medicine, Marshfield Clinic, Marshfield, WI.
Abstract
OBJECTIVES: To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH). METHODS: We reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes. RESULTS: A total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission. CONCLUSIONS: DAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.
OBJECTIVES: To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH). METHODS: We reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes. RESULTS: A total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission. CONCLUSIONS:DAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.
Authors: Anne Angelillo-Scherrer; Behrouz Mansouri Taleghani; Frauke Förger; Gabriela M Baerlocher; Thomas Pabst; Alexander Pöllinger; Yara Banz; Thomas Geiser; Johanna A Kremer Hovinga; Alicia Rovó Journal: Blood Adv Date: 2019-09-10
Authors: Jae Won Kim; Tae Woo Kim; Keon Hee Ryu; Sun Gyoo Park; Chang Young Jeong; Dong Ho Park Journal: J Int Med Res Date: 2020-01 Impact factor: 1.671
Authors: Adrien Mirouse; Antoine Parrot; Vincent Audigier; Alexandre Demoule; Julien Mayaux; Guillaume Géri; Eric Mariotte; Nicolas Bréchot; Nicolas de Prost; Mathieu Vautier; Mathilde Neuville; Naïke Bigé; Etienne de Montmollin; Patrice Cacoub; Matthieu Resche-Rigon; Jacques Cadranel; David Saadoun Journal: Crit Care Date: 2020-05-18 Impact factor: 9.097
Authors: Hannah Cohen; Maria J Cuadrado; Doruk Erkan; Ali Duarte-Garcia; David A Isenberg; Jason S Knight; Thomas L Ortel; Anisur Rahman; Jane E Salmon; Maria G Tektonidou; David J Williams; Rohan Willis; Scott C Woller; Danieli Andrade Journal: Lupus Date: 2020-10 Impact factor: 2.911