TABLE 1 Diseases Associated with Relapsing Polychondritis

Autoimmune rheumatic diseases Rheumatoid arthritis Juvenile idiopathic arthritis Systemic lupus erythematosus Progressive systemic sclerosis Sjögren's syndrome Mixed connective tissue disease Ankylosing spondylitis Psoriatic arthritis Reiter's syndrome RS3PE

Hematological disorders Myelodysplastic syndromes Hodgkin's disease MALT-type lymphoma Non-Hodgkin's lymphomas Acute lymphoblastic leukemia Pernicious anemia

Endocrine diseases Diabetes mellitus type 1 Hashimoto's thyroiditis Graves' disease

Vasculitides Leucocytoclastic Wegener's granulomatosis Polyarteritis nodosa Microscopic polyangiitis Churg-Strauss syndrome Behçet's disease and MAGIC syndrome Mixed cryoglobulinemia

Gastrointestinal disease Crohn's disease Ulcerative colitis Primary biliary cirrhosis

Other conditions Retroperitoneal fibrosis Myasthenia gravis Pyoderma gangrenosum

Psoriasis vulgaris Chondrosarcoma

Abbreviations: MAGIC, mouth and genital ulcers with inflamed cartilage; MALT, mucosa-associated lymphoid tissue; RS3PE, remitting seronegative symmetrical synovitis with pitting edema.

Biopsy of inflamed auricular tissue shows loss of basophilic staining of the cartilage matrix associated with perichondral inflammation at the cartilage-soft-tissue interface (8). The perichondral infiltrate is primarily lymphocytes (CD4+ more than CD8+ T cells), with variable numbers of polymorphonuclear cells, monocytes/macrophages, and plasma cells. Granular deposits of IgG and C3 can be found at the interface by direct immunofluores-cence. Later, with disease progression, chondrocytes degenerate, the cartilage matrix becomes further depleted, and nonspecific granulation tissue invades the cartilage (Fig. 1). Eventually, there is a complete disruption of the cartilage architecture, with fibrosis and focal areas of calcification.



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