Intrapulmonary hemorrhage in collagen-vascular diseases includes a spectrum of underlying conditions

To elucidate the background and clinical features of intrapulmonary hemorrhage in collagen-vascular diseases (CVD) patients.

The charts of collagen-vascular diseases patients who were hospitalized and had intrapulmonary hemorrhages between 1981 and 2006 were retrospectively examined for underlying diseases, clinical and laboratory features, and treatments and outcomes.

Of 4,017 patients, 11 females aged 52.1+/-12 had total of 17 episodes of diffuse or non-diffuse intrapulmonary hemorrhage. Fourteen episodes of diffuse alveolar hemorrhage (DAH) developed in 4 microscopic polyangiitis (MPA) patients having a high MPO-ANCA level, 4 systemic lupus erythematosus (SLE) patients having a high SLEDAI score, and 1 SLE/MPA patient having a high MPO-ANCA level. Among the 9 DAH patients, 2 had complicated Goodpasture syndrome, 3 had thrombotic thrombocytopenic purpura (TTP), and 1 had disseminated intravascular coagulation. In DAH the peripheral blood hemoglobin level decreased from 9.3+/-2.2 (n=13) to 6.8+/-1.5 g/dL (n=14, p<0.0001) at 0.5+/-0.7 g/dL/day, and the lymphocyte count decreased from 854+/-424 to 462+/-376 /microL. No patient died of DAH, including 1 who spontaneously remitted. The 3 episodes of non-DAH included 2 pulmonary aneurysm ruptures in 1 SLE patient, and 1 thromboembolism that developed in 1 SLE patient who had anti-phospholipid antibody; their SLEDAI scores were low and these remitted spontaneously.

Of intrapulmonary hemorrhage in CVD patients, DAH developed with active MPA or SLE, upon which Goodpasture syndrome or TTP was occasionally superimposed. With DAH, the magnitude of peripheral blood Hb level decrease was approximately 0.5 g/dL/day, and the lymphocyte count decreased. No patient died of DAH.