PURPOSE: Diffuse Alveolar Hemorrhage (DAH) is considered to be a catastrophic manifestation of Systemic Lupus Erythematosus (SLE), but rarely be an important clinical feature of other Collagen Vascular Diseases (CVD). The purpose of this study is to determine the frequency, histological categorization and clinical characteristics of DAH in patients died as a consequence of SLE and other CVD.
METHODS: A number of 3,400 complete autopsies carried out between 1958 and 2006 were reviewed, in search of patients with SLE and other CVD diagnosis. Potential cause of death was determined by clinical and histological data. DAH was considered when it was the primary pathological finding in the autopsy.
RESULTS: A total of 90 SLE and 26 nonSLE-CVD patients were included. Twenty-three SLE patients showed DAH in the autopsy, diffuse in 21 and patchy in 2. In 15 patients pulmonary infection coexisted. In only 3 patients the histological pattern was capillaritis, all showing necrotizing pulmonary capillaritis and acute and chronic alveolar hemorrhage. In only one DAH was considered the direct cause of death. Four non-SLE CVD patients showed alveolar hemorrhage (15, 4%). None of them demonstrated capillaritis, or renal involvement. When patients with DAH were compared, SLE patients were younger (p= 0,037), and had shorter time from the onset of the disease (p = 0,015) but they were not difference in any other of the analyzed clinical variables.
CONCLUSION: The presence of DAH in critically patients with CVD is not unusual but it is often seen concomitantly with other pulmonary manifestations. Pulmonary capillaritis is infrequently found in SLE patients and we have not recorded any case in non-SLE patients.
CLINICAL IMPLICATIONS: In critically ill CVD patients, DAH is not infrequent and is mainly associated to infection. No clinical prediction of the histological pattern of DAH is possible and from a clinical point of view patients should be considered as having a potential immunologically mediated disease and concomitant pulmonary infection.
DISCLOSURE: Lorena Delisio, No Financial Disclosure Information; No Product/Research Disclosure Information