Mixed Cryoglobulinemia Syndrome (MCS) due to untreated hepatitis B with uncommon presentation: case report and literatur
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BMC Rheumatology
CASE REPORT
Open Access
Mixed Cryoglobulinemia Syndrome (MCS) due to untreated hepatitis B with uncommon presentation: case report and literature review Nasam Alfraji1,2* , Vandan D. Upadhyaya1, Christopher Bekampis1 and Halyna Kuzyshyn3
Abstract Background: The mixed cryoglobulinemia (MC) syndrome is a systemic inflammatory syndrome that causes smallto-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. Keywords: Pulmonary alveolar hemorrhage, Mixed cryoglobulinemia, Vasculitis, Hepatitis B virus
Background Serum cryoglobulins are found in a wide variety of disorders [1]. However, majority of people with cryoglobulins can be asymptomatic and their presence can carry no clinical significance [1]. Symptoms and clinical findings are correlated with the underlying Brouet type of cryoglobulin - Type I, II, or III * Correspondence: [email protected] 1 Department of Medicine, Jersey Shore University Medical Center, Neptune, NJ 07753, USA 2 Internal Medicine Residency Program, Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ 07753, USA Full list of author information is available at the end of the article
[1, 2]. Cryoglobulins type I are usually associated with lymphoproliferative disorders, while type II and III (mixed cryglobulins) are associated with infections, and connective tissue/autoimmune diseases [1–5]. Pulmonary involvement in MC is a rare but reported finding [6]. Alveolar hemorrhage has been noted in up to 3.2% of cryoglobulinemia cases, and most often been associated with hepatitis C antibodies [6–9]. Initially such cases were often mistaken for severe pneumonia, but persistent interstitial infiltrates and hemosiderinladen macrophages in bronchoalveolar lavage fluid started to suggest otherwise [9]. Such features of pulmonary vasculitis are rarely seen in MC especially in
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