AB1651 DIFFUSE ALVEOLAR HEMORRHAGE IN RHEUMATOLOGY: CLINICAL PRESENTATION, ETIOLOGY, TREATMENT AND OUTCOME

Abstract

BackgroundDiffuse alveolar hemorrhage (DAH) is characterized by rapid onset of pulmonary infiltrates and hypoxemia. It is a potentially life threatening complication associated with systemic autoimmune conditions.ObjectivesTo describe the presentation, clinical features, etiology, treatment and outcome of DAH in rheumatological conditions.MethodsThis a retrospective, multicentre study involveing 3 rheumatology centers, wherein data about DAH cases seen over the past 5 years was collected.ResultsThere were 19 patients (Female = 15). The mean age was 38 years (18 - 67). DAH was the presenting feature in 15 patients (78%). The mean duration of symptoms was 20 days (1 - 60). The symptoms of DAH were: dyspnea 16 (84%), cough 15 (78%), hemoptysis 13 (68%). Other features of DAH like hypoxemia (18 patients), fall in hemoglobin (19 patients) were common. Fever was observed in 9 (47%). Nephritis was noted in 14 (73%). Renal biopsy was done in 6 (Lupus nephritis class 3 & 5 = 1; lupus nephritis class 4 = 1; Pauci immune crescentic glomerulonephritis = 3;Anti GBM associated crescentic glomerulonephritis =1). The most common feature on chest radiograph was diffuse opacities (Image 1). The etiology of DAH was as follows (Table 1) - granulomatosis with polyangiitis (GPA = 8); Systemic lupus erythematosus (SLE =7); anti GBM disease (2); Microscopic polyangiitis (MPA=1) and undifferentiated vasculitis [1]. The treatment of DAH included - Intravenous methylprednisolone (IV MP)=18; Intravenous immunoglobulin (IVIG) = 2; Extracorporeal membrane oxygenation (ECMO) = 1; Therapeutic plasma exchange = 3; Intravenous cyclophosphamide = 11; Rituximab (RTX) = 4; Mycophenolate mofetil (MMF) = 1. Most of the patients were managed in the intensive care unit (ICU) (13/ 19) and needed ventilatory support (11/ 19). Most patients required more than 10 days of hospitalization (12/ 19 = 63%). There were a total of 5 deaths (GPA =2; SLE =2; anti-GBM disease =1).ConclusionDAH was the presenting feature in most of the cases. GPA and SLE were the common causes. Dyspnea, cough and Hemoptysis were the common DAH symptoms. Fever and nephritis were the common Non - DAH clinical feature. Most of the patients were treated with IV MP and required ICU care; Approximately half of the patients required ventilatory support. DAH is a potentially life threatening condition with an overall mortality rate of approximately 25%.Early recognition and appropriate treatment is needed for better outcome.Reference[1]Park, M. S. (2013). Diffuse alveolar hemorrhage.Tuberculosis and Respiratory Diseases,74(4), 151–162.https://doi.org/10.4046/trd.2013.74.4.151Table 1.DAH - Causes, clinical features, treatment and outcomeDiagnosisDAH featuresNon DAH featuresTreatmentOutcomeGPA (N=8)(M/ F= 4/4)DAH as presenting feature = 6Cough - 5Dyspnea - 6Hemoptysis - 3Nephritis - 5Fever - 4LRT - 3Skin rash - 2ENT - 1Oral ulcers - 1Scleritis – 1IV MP - 8TPE - 3IV CYC - 6RTX - 2ICU - 5Ventilatory support - 4Discharge within days - 2Discharge after 10 days - 5Death - 2SLE (N=7)(F = 6)DAH as presenting feature = 3Cough - 6Dyspnea - 6Hemoptysis - 6Nephritis - 6ITP - 5Fever - 5Alopecia - 2Oral ulcers - 2AIHA - 2CNS - 1Serositis – 1IV MP - 6IVIG - 2IV CYC - 2RTX - 2ICU - 5Ventilatory support - 4Discharge within days - 1Discharge after 10 days - 4Death - 2Anti-GBM disease (N = 2; All female)DAH at presentation = 2Cough - 2Dyspnea - 2Hemoptysis - 2Nephritis - 2Arthritis – 1IV MP - 2TPE -IVIG -IV CYC - 1RTX -ECMO - 1ICU - 2Ventilatory support - 2Discharge after 10 days - 1Death - 1MPA (N=1)(Female)DAH at presentation = YCough - YDyspnea - YHemoptysis - YNephritis – 1IVMP - YIV CYC - YICU - NVentilatory support- NDischarge after 10 days - 1Undifferentiated vasculitis (N = 1)(Female)DAH at presentation = YCough - YDyspnea - YHemoptysis - YSkin rashIVMP - YIV CYC - YTPE - YICU - YVentilatory support - YOngoing treatment> 10 days of hospitalizationLRT - lower respiratory tract; ENT - Ear, nose, throat; AIHA - Autoimmune hemolytic anemia; ITP - Idiopathic thrombocytopenic purpura; CNS - Central nervous system; M - Male; F - Female: Y = Yes;N = No.Image 1: Chest X rayAcknowledgements:NIL.Disclosure of InterestsNone Declared.