Background/Purpose: pulmonary hemorrhage (PH) may complicate systemic disease. The main objective of this study was to describe characteristics, treatment and outcome of patients presenting severe PH related to systemic disease.

Methods: we performed a French multicentric retrospective study including all cases of adult patients presenting severe PH related to systemic disease from 2000 to 2016. Severe PH was defined as PH requiring intensive care unit (ICU) admission. Data concerning clinical, biological and radiological findings were collected, as well as treatments.

Results: we identified 70 patients (52.9% men) admitted with severe PH due to a systemic disease with a median age of 48 (29.8-66.3), in 7 centers. Among these patients, 21 (30%) had a past medical history of systemic disease. Main symptom for consultation was dyspnea in 69 (98.6%) cases. Patients were hospitalized 27.5 (10-66) days after the first symptoms and 6.5 (1-29) days after the beginning of respiratory symptoms. There was evidence a pulmonary-renal syndrome in 54 (78.3%) cases, articular manifestations in 9 (13.0%) cases, skin manifestations in 16 (23.2%) cases, nervous manifestations in 12 (17.4%) cases, and digestive manifestations in 5 (7.2%) cases. ICU admission occurred 4 (1-11.5) days after hospital admission. All patients presented anemia with a median hemoglobin level of 8.2 (6.9-9.4) g/dl. Median blood creatinine level was 222 (91-429) µmol/l. Chest X-ray was normal in 3 (4.6%) patients, there was bilateral alveolar condensations in 51 (78.5%) patients and bilateral interstitial pattern in 28 (43%) patients. CT-scan was realized for 50 (71.4%) patients and was never normal. Main findings included ground glass opacities in 44 (88%) cases, nodules in 11 (22%) cases, and a pleural effusion in 6 (12%) cases. Sixty-one (87.1%) patients underwent a bronchial fibroscopy showing PH for all patients (100%). Diagnosis was made 31 (10.5-62.5) days after the first symptoms and 5 (2-10) days after hospitalization. A vasculitis diagnosis was made for 40 (57.1%) patients with ANCA, IgA and cryoglobulinemic vasculitis in 33 (47.1%), 3 (5.7%), and 4 (5.7%) cases, respectively. A connective tissue disorder was diagnosed for 21 (30%) patients, mainly with lupus and antiphospholipid syndrome (APS) in 9 (12.9%) and 6 (8.6%), respectively. Anti-MBG disease (Goodpasture syndrome) was diagnosed in 9 (12.9%) cases. Steroids were used in 69 (98.6%) patients with bolus in 61 (87.1%) patients. Cyclophosphamide and Rituximab were used for 49 (70%) and 8 (11.4%) patients, respectively. Plasmapheresis was used in 38 (54.3%) patients. During ICU stay, 67 (95.7%) patients required oxygen therapy, 34 (48.6%) were intubated with a median ventilation length of 12 (6.3-25.3) days, 40 (57.1%) required renal replacement therapy, and 25 (35.7%) vasopressive drugs. Median ICU and hospital length of stay were 11 (6.8-19.3%) and 39 (21.3-64) days. Twelve (17.1%) patients died. After a median follow-up of 7.5 (2-34) months, 17 (39.5%) patients required renal replacement therapy, and 3 (7.6%) had a chronic lung disease.

Conclusion: PH is a serious complication of systemic disease accounting for 17% of deaths. Even if the use of steroids, immunosuppressive therapy, and plasmapheresis seem to be effective, the best regimen remains to be determined.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-treatment-and-outcome-of-severe-pulmonary-hemorrhage-related-to-systemic-disease-french-multicentric-study/