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Cryoglobulinemia due to Hepatitis C with Pulmonary Renal Syndrome

DOI: 10.1155/2013/278975

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Abstract:

Cryoglobulinemia is an uncommon condition typically due to hepatitis C infection. Its clinical presentation is varied and often reflects deposition of immune complex and complement deposition. Renal compromise is observed in approximately one third of patients with mixed cryoglobulinemia and reports of concomitant pulmonary involvement are quite rare. We report a case of a patient who presented with pulmonary and renal manifestations of cryoglobulinemia with a serum rheumatoid factor over one hundred times the upper limit of normal and benefited from high-dose steroids and plasmapheresis in the acute setting. 1. Background Cryoglobulinemia is a rare entity typically due to hepatitis C infection whose widely variable presentation can make prompt and accurate diagnosis difficult. Many of the features of cryoglobulinemia are manifestations of immune complex and complement deposition in the small- and medium-sized vessels [1–4]. The resultant vasculitis is observed in multiple organ systems with considerable variability between patients. The most common clinical features include purpura, weakness, and arthralgias; all of which are experienced by a vast majority of patients although perhaps only intermittently. Renal involvement is witnessed in 31% of patients with mixed cryoglobulinemia, and overall prognosis is worse for such patients [1]. Again, there is considerable variance in the severity of disease, from those who remain asymptomatic to those progressing to chronic renal insufficiency [4]. Pulmonary involvement in cryoglobulinemia is rare but had been documented as early as 1979 [5]. Typically, the patient has no signs or symptoms of lung involvement. Very rarely, the patient with cryoglobulinemia has concomitant alveolar hemorrhage. Ten case reports with such features have been published, and the presence of alveolitis seemed to portend a very poor prognosis. Of note, nine of those ten patients were also in renal failure at the time of alveolar hemorrhage. There was no specific therapeutic option that was shown to be of clear benefit. One patient did experience remission with plasmapheresis; however, five others did not [3]. 2. Case Report A forty-nine year old male presented to the emergency department complaining of hemoptysis of one-month duration. Past medical history included untreated hypertension and an episode of pericarditis two years earlier. The patient endorsed a 35-pack-year smoking history and consumption of 12 alcoholic beverages daily in addition to a remote history of intravenous drug use. Significant physical exam findings included

References

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[2]  M. Prasad, G. K. Buller, C. I. Mena, and A. N. Sofair, “Sum of the parts,” The New England Journal of Medicine, vol. 355, no. 23, pp. 2468–2473, 2006.
[3]  H. Amital, A. Rubinow, and Y. Naparstek, “Alveolar hemorrhage in cryoglobulinemia—an indicator of poor prognosis,” Clinical and Experimental Rheumatology, vol. 23, no. 5, pp. 616–620, 2005.
[4]  E. D. Charles and L. B. Dustin, “Hepatitis C virus-induced cryoglobulinemia,” Kidney International, vol. 76, no. 8, pp. 818–824, 2009.
[5]  S. Bombardieri, P. Paoletti, and C. Ferri, “Lung involvement in essential mixed cryoglobulinemia,” American Journal of Medicine, vol. 66, no. 5, pp. 748–756, 1979.
[6]  F. Fabrizi, G. Lunghi, P. Messa, and P. Martin, “Therapy of hepatitis C virus-associated glomerulonephritis: current approaches,” Journal of Nephrology, vol. 21, no. 6, pp. 813–825, 2008.

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