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Vasculitis secondary to cryoglobulinemia occurs in only a small minority of patients with cryoglobulins in their serum, but this diagnosis should be considered when patients present with palpable purpura, especially when recurrent, and peripheral neuropathy A proliferative glomerulonephritis that strongly resembles lupus nephritis may complicate cryoglobulinemia Abnormal liver function tests, abdominal pain, and pulmonary disease may also occur The diagnosis is based on a compatible clinical picture and a positive serum test for cryoglobulins Type I cryoglobulins (monoclonal proteins that lack rheumatoid factor activity) are more commonly seen in lymphoproliferative disease (Type I cryoglobulins usually cause hyperviscosity syndromes rather than vasculitis) The diseases most commonly associated with cryoglobulinemic vasculitis are hepatitis C and connective tissues diseases, especially Sj gren s syndrome Type II (monoclonal antibody with rheumatoid factor activity) and Type III (polyclonal antibody with rheumatoid factor activity) cryoglobulins cause vasculitis Because 90% of cryoglobulinemia cases are associated with hepatitis C infections, the optimal approach to treatment is viral suppression with interferon- with or without ribavirin Pegylated forms of interferon- are now the standard of care for mixed cryoglobulinemia because this form of the drug requires less frequent administration and is more effective Because immunosuppressive agents may facilitate viral replication, corticosteroids, cyclophosphamide, and other interventions (including plasmapheresis) should be reserved for organthreatening complications B cell depletion using agents such as rituximab appears to be a promising avenue of therapy
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Kamar N et al Treatment of hepatitis C-virus-related glomerulonephritis Kidney Int 2006 Feb;69(3):436 9 [PMID: 16514428] Quartuccio L et al Rituximab treatment for glomerulonephritis in HCV-associated mixed cryoglobulinaemia: efficacy and safety in the absence of steroids Rheumatology (Oxford) 2006 Jul;45(7):842 6 [PMID: 16418196] Saadoun D et al Antiviral therapy for hepatitis C virus associated mixed cryoglobulinemia vasculitis: a long-term followup study Arthritis Rheum 2006 Nov;54(11):3696 706 [PMID: 17075881]
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RELAPSING POLYCHONDRITIS
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This disease is characterized by inflammatory destructive lesions of cartilaginous structures, principally the ears, nose, trachea, and larynx Nearly 40% of cases are associated with another disease, especially either other immunologic disorders (such as SLE, rheumatoid arthritis, or Hashimoto s thyroiditis) or cancers (such as multiple myeloma) or hematologic disorders (such as myelodysplastic syndrome) The disease, which is usually episodic, affects males and females equally The cartilage is painful, swollen, and tender during an attack and subsequently becomes atrophic, resulting in permanent deformity Biopsy of the involved cartilage shows inflammation and chondrolysis Noncartilaginous manifestations of the disease include fever, episcleritis, uveitis, deafness, aortic insufficiency, and rarely glomerulonephritis In 85% of patients, a migratory, asymmetric, and seronegative arthropathy occurs, affecting both large and small joints and the costochondral junctions Diagnosing this uncommon disease is especially difficult since the signs of cartilage inflammation (such as red ears or nasal pain) may develop and be more subtle than the fever, arthritis, rash, or other systemic manifestations Prednisone, 05 1 mg/kg/d orally, is often effective Dapsone (100 200 mg/d orally) or methotrexate (75 20 mg orally per week) may also be effective, sparing the need for long-term high-dose corticosteroid treatment Involvement of the tracheobronchial tree, leading to tracheomalacia, may lead to difficult management issues
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Dib C et al Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the Mayo Clinic records Mayo Clin Proc 2006 Jun;81(6):772 6 [PMID: 16770977]
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Hen ch-Sch nlein purpura, the most common systemic vasculitis in children, occurs in adults as well Typical features are palpable purpura, abdominal pain, arthritis, and hematuria Pathologic features include leukocytoclastic vasculitis with IgA deposition The cause is not known The purpuric skin lesions are typically located on the lower extremities but may also be seen on the hands, arms, trunk, and buttocks Joint symptoms are present in the majority of patients, the knees and ankles being most commonly involved Abdominal pain secondary to vasculitis of the intestinal tract is often associated with gastrointestinal bleeding Hematuria signals the presence of a renal lesion that is usually reversible, although it occasionally may progress to renal insufficiency Children tend to have more frequent and more serious gastrointestinal vasculitis, whereas adults more often suffer from renal disease Biopsy of the kidney reveals segmental glomerulonephritis with crescents and mesangial deposition of IgA
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