Relapsing polychondritis, chronic hepatitis C virus infection, and mixed cryoglobulemia

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Abstract

Objective

Review of relapsing polychondritis (RP) and its association to chronic hepatitis C virus (HCV) infection and mixed cryoglobulinemia.

Methods

A case of RP associated with HCV infection is reported. The English language medical and scientific literature was reviewed for RP, hepatitis C, and its relation to other connective tissue diseases from February 1966 to January 2003 using MEDLINE.

Results

RP is an uncommon, multisystem disease of unknown etiology characterized by recurrent inflammation of cartilaginous and related tissues, being associated with other diseases in 30% to 35% of cases. HCV infection is a systemic illness with a propensity to trigger or exacerbate autoimmune disorders: eg, essential mixed cryoglobulinemia, membranoproliferative glomerulonephritis, and leukocytoclastic and systemic vasculitis. We could find no previous report of an association between RP with HCV and mixed cryoglobulinemia. Treatment with interferon gamma and ribavirin (IR) not only induced an undetectable viral load, but also resolved symptoms of RP.

Conclusions

We report a patient with RP, HCV, and mixed cryoglobulinemia. It is unknown if there is a cause-effect or chance relationship. Treatment with IR improved the symptoms of RP. It is not known whether the effects of IR were directly on the RP or suppressed RP indirectly through the actions on the viral load or active hepatitis.

Section snippets

Methods

A case of RP associated with HCV infection is reported. The English language medical literature for RP as well as hepatitis C and cryoglobulinemia associated with polychondritis were reviewed from February 1966 to January 2003 using MEDLINE. Relevant articles published in peer-reviewed journals were analyzed. References of reviewed studies were screened for additional references not apparent from the search. Reviewed studies included those involving history, epidemiology, immunologic mechanisms

Case report

A 74-year-old woman with a medical history of acute myelocytic leukemia 11 years previously was effectively treated and in remission by bone marrow biopsy. One year after the diagnosis of leukemia, she was found to have liver enzymes elevated twice the upper limits of the normal range. Liver biopsy revealed severe portal fibrosis with cholangitis and hemosiderosis. The abnormal liver disease was felt to be the result of leukemia-related multiple blood transfusions. After 10 years, the patient

Epidemiology

The peak age of RP onset is in the fourth and fifth decades, but it has been reported in both extremes of life. The youngest case reported was in a newborn (11), and the oldest was in the ninth decade (12). The annual incidence rate is 3.5 per million. Most authors report an equal gender distribution; however, women develop airway complications more frequently 1, 13, 14. When myelodysplastic syndromes are associated with RP, men predominate (15). All races are affected, with the greatest number

Diagnosis and investigations of RP

The diagnosis of RP is frequently overlooked and delayed because of its low incidence and uncommon symptoms. Several conditions may mimic the clinical presentation, such as infections, allergic reactions, trauma, tumor, and granulomatous diseases. Trentham et al (3) reported a mean delay of 2.9 years from the time that medical attention was sought until diagnosis was established. The delay was longer than 1 year for 68% of patients, and one third of patients presented to 5 or more physicians

Discussion

RP is a rare disease characterized by episodes of inflammation of cartilaginous tissues. HCV infection is more common and often exhibits extrahepatic manifestations, such as mixed cryoglobulinemia. We could find no previous report of RP associated with HCV and mixed cryoglobulinemia. Our patient presented with bilateral auricular chondritis, nasal chondritis, and response to steroids, thus fulfilling the criteria of RP of Damiani and Levine (92). Within 1 year, she also was diagnosed with HCV

Ivonne Herrera, MD: Fellow in Rheumatology and Immunology, Department of Medicine, University of Miami School of Medicine, Miami, Florida

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    Ivonne Herrera, MD: Fellow in Rheumatology and Immunology, Department of Medicine, University of Miami School of Medicine, Miami, Florida

    Ronald Concha, MD: Resident in Internal Medicine, Department of Medicine, University of Miami School of Medicine, Miami, Florida

    Enrique G. Molina, MD: Assistant Professor of Medicine, Center for Liver Diseases, Department of Medicine, University of Miami School of Medicine, Miami, Florida

    Eugene R. Schiff, MD: Professor of Medicine and Chief, Division of Hepatology Center for Liver Diseases, Department of Medicine, University of Miami School of Medicine, Miami, Florida

    Roy D. Altman, MD: Clinical Director, GRECC, Miami VAMC, Professor of Medicine and Chief, Rheumatology and Immunology, Department of Medicine, University of Miami School of Medicine, Miami, Florida.

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