Relapsing polychondritis is an uncommon disorder of unknown cause characterized by inflammation of cartilage predominantly affecting the ears, nose, and laryngotracheobronchial tree. Multisystem disease occurs commonly and can also involve noncartilaginous tissues and organs. Relapsing polychondritis has been estimated to have an annual incidence of 3.5 per million. The peak age of onset is between 40 and 50 years, but the disease can be seen in all ages with both sexes being equally affected. Approximately 30% of patients with relapsing polychondritis will have another rheumatologic disorder, most frequently systemic vasculitis, rheumatoid arthritis, or systemic lupus erythematosus (SLE). Nonrheumatic disorders have also been associated with relapsing polychondritis (Table 366-1). In most cases, these disorders antedate the appearance of relapsing polychondritis, usually by months or years; however, in other instances, the onset of relapsing polychondritis can accompany disease presentation.
TABLE 366-1Disorders Associated with Relapsing Polychondritisa ||Download (.pdf) TABLE 366-1 Disorders Associated with Relapsing Polychondritisa
|Systemic vasculitis |
|Rheumatoid arthritis |
|Systemic lupus erythematosus |
|Overlapping connective tissue disease |
|Behçet’s disease |
|Polymyalgia rheumatica |
|Primary biliary cirrhosis |
|Pulmonary fibrosis |
|Hashimoto’s thyroiditis |
|Graves’ disease |
|Crohn’s disease |
|Ulcerative colitis |
|Myelodysplastic syndrome |
PATHOLOGY AND PATHOPHYSIOLOGY
The earliest abnormality of hyaline and elastic cartilage noted histologically is a focal or diffuse loss of basophilic staining indicating depletion of proteoglycan from the cartilage matrix. Inflammatory infiltrates are found adjacent to involved cartilage and consist predominantly of mononuclear cells and occasional plasma cells. In acute disease, polymorphonuclear white cells may also be present. Destruction of cartilage begins at the outer edges and advances centrally. There is lacunar breakdown and loss of chondrocytes. Degenerating cartilage is replaced by granulation tissue and later by fibrosis and focal areas of calcification. Small loci of cartilage regeneration may be present. Extracellular granular material observed in the degenerating cartilage matrix by electron microscopy has been interpreted to be enzymes, immunoglobulins, or proteoglycans.
The available data suggest that both humoral and cell-mediated immunity play an important role in the pathogenesis of relapsing polychondritis. Immunoglobulin and complement deposits are found at sites of inflammation. In addition, antibodies to type II collagen and to matrilin-1 and immune complexes are detected in the sera of some patients. The possibility that an immune response to type II collagen may be important in the pathogenesis is supported experimentally by the occurrence of auricular chondritis in rats immunized with type II collagen. Antibodies to type II collagen are found in the sera of these animals, and immune deposits are detected at sites of ear inflammation. Humoral immune responses to type IX and type XI collagen, matrilin-1, and cartilage oligomeric matrix protein have been demonstrated in some patients. Matrilin-1 is a noncollagenous protein ...