Mixed Cryoglobulinemia Syndrome (MCS) due to untreated hepatitis B with uncommon presentation: case report and literature review

Abstract

Background The mixed cryoglobulinemia (MC) syndrome is a systemic inflammatory syndrome that causes small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes most commonly caused by chronic hepatitis C virus (HCV), and rarely by chronic hepatitis B virus (HBV). Its clinical presentation is significantly varied, with manifestations ranging from purpura, arthralgia, and myalgia to more severe neurologic and renal involvement. Pulmonary involvement as organizing pneumonia, alveolar hemorrhage, and pulmonary vasculitis have been reported, but appear to be quite rare. Case presentation We report an uncommon case of a patient who presented with primary pulmonary syndrome without renal involvement in the setting of MC, due to untreated chronic hepatitis B infection. Early diagnosis and consequent institution of glucocorticoids, B-cell-depleting monoclonal antibody and antiviral therapy led to a favorable outcome and prevented any fatal sequelae. Conclusion Pulmonary compromise in MC syndrome is very uncommon and carries a high rate of mortality. Therefore, in patients with HBV presenting with hemoptysis, physicians must carry a high clinical suspicion for alveolar hemorrhage secondary to cryoglobulinemic vasculitis.

The Role of B Cells in Adult and Paediatric Liver Injury

“…B cells contribute to the formation of cryoglobulins through uncontrolled autoantibody production and proliferation ( 149 ). These cryoglobulin-containing immune complexes deposit in small or medium vessels causing vasculitis ( 145 , 150 , 151 ). Whilst cryoglobulinemia is common in HCV, rare cases have been reported to exist in HBV infected patients ( 151 ).…”

“…These cryoglobulin-containing immune complexes deposit in small or medium vessels causing vasculitis ( 145 , 150 , 151 ). Whilst cryoglobulinemia is common in HCV, rare cases have been reported to exist in HBV infected patients ( 151 ). The clonal proliferation of B cells in MC ( 152 ), may cause the formation of ectopic lymphoid aggregates within the liver of HCV patients.…”