Case Presentation: A 51 year-old man with untreated HCV (HCV-RNA 460978 IU/ML, Genotype 3a), prior heroin abuse on methadone maintenance presented with hematuria and right upper quadrant pain for 2 days. The exam was significant for diffuse abdominal discomfort and no evidence of ascites. Initial labwork showed stable normocytic anemia and hepatic function compared to prior values. Amylase and lipase were normal. Cr 1.54 MG/DL was elevated from normal values 4 months earlier. Urinalysis revealed 30 protein and 19 RBC. Abdominal CT suggested cirrhosis and splenomegaly. A HIDA scan was negative. Despite medical therapy, the abdominal pain persisted and non-oliguric acute kidney injury developed with peak Cr 2.71 MG/DL. Repeat urinalysis showed worsening hematuria and proteinuria and a spot urine protein/creatinine ratio revealed nephritic syndrome. He developed new microcytic anemia (HB 7 G/DL, MCV 78 FL) followed by a palpable purpuric rash on the extremities with spread to his face and trunk. Additional workup suggested mixed cryoglobulinemia: isolated low C4, elevated serum IgM, elevated RF, positive serum cryoglobulin and negative ANA. Renal biopsy showed membranoproliferative glomerulonephritis with cryoglobulin deposits. Treatment with plasmapheresis and intravenous pulsed steroids improved the renal function to Cr 0.9 MG/DL. Shortly after discharge, he was readmitted for recurrent episodes of diffuse pulmonary alveolar hemorrhage requiring intubation and plasmapheresis. His clinical course eventually stabilized and he was initiated on Ribavarin and Sofosbuvir with good viremic response to date (HCV-RNA undetectable IU/ML). The most recent renal function is stable at Cr 1.31 MG/DL and his hemoglobin improved to 11.1 G/DL.
Discussion: This case highlights the importance of a high index of suspicion of acute cryoglobulinemia in patients with HCV and unexplained symptoms even when they precede cutaneous manifestations, which are almost always present. Additional manifestations include arthalgias, myalgias, fatigue, peripheral neuropathy, pulmonary infiltrates and renal disease. Renal involvement manifests early and with an incidence of up to 60%. Failure to treat may result in renal failure and those with HCV-associated disease appear to be at greater risk. Prompt diagnosis and control of the immune phenomena with immunosuppressive medications and/or plasmapheresis and early antiviral treatment of HCV is recommended. Prognosis in patients with HCV and mixed cryoglobulinemia correlates with response to antiviral therapy.
Conclusions: While cryoglobulins are present in more than 30% of patients with chronic HCV, the clinical syndrome of acute cryoglobulinemia remains a diagnostic challenge due to its rarity and diverse manifestations. Our case demonstrates that nonspecific symptoms may precede the common cutaneous findings. Early recognition and treatment is essential in preventing catastrophic outcomes.
To cite this abstract:Galal N, Kato H, Dharapak P. Expecting the Expected at an Unexpected Time. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 522. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/expecting-the-expected-at-an-unexpected-time/. Accessed September 16, 2019.