Alveolar hemorrhage in cryoglobulinemia--an indicator of poor prognosis

Alveolar vasculitis is an unusual event in the course of cryoglobulinemia (CG). The inflammatory process involving the alveolar capillary walls may result in severe alveolar hemorrhage and consequently lead to a grave outcome. The objective of this study was to evaluate the occurrence of this unusual finding in CG.

We reviewed the records of all patients with CG who developed acute alveolitis, registered their associated clinical and laboratory parameters and evaluated the possible impact these parameters may have on their prognosis. In addition we scanned the Medline for similar cases.

Of the 125 patients with CG who were hospitalized in our medical center during the last 23 years, 4 (3.2%) developed alveolar hemorrhage. All patients exhibited extreme fatigue, fever with clinical and radiological evidence of alveolitis. Of the 4 new cases, 1 had type II CG and 3 had type III CG. Of our 4 patients, 3 developed concomitant acute renal failure necessitating hemodialysis. A literature survey resulted in 6 additional cases. All 10 patients experienced acute respiratory insufficiency and eight had at least one episode of hemoptysis. In the other 2 patients the bronchoalveolar lavage (BAL) fluid contained hemosiderin laden macrophages. Five of the 10 patients had concomitant hepatitis C virus (HCV) infection; 2 patients were seen prior to modern identification of the HCV; however, liver abnormalities were not described. Of the 10 patients 5 patients had type II CG and 5 others had type III. Of the 7 patients in whom outcome was available, 6 died from their illness. Acute renal failure or exacerbation of antecedent glomerular disease occurred in 8 patients.

Alveolitis is a rare manifestation of CG, presenting as an overwhelming systemic illness and portends a poor prognosis with a high mortality rate.