Relapsing polychondritis

Relapsing polychondritis (RP) is a rare multisystem disease with progressive inflammatory destructive lesions that relapse and remit, involving cartilage and other connective tissues including ear, nose, eye, joints, respiratory tract and cardiovascular system. Clinically it shows chondritis of the ear, nose and respiratory tract with eye and vestibular involvement; polyarthritis and vascular involvement are also common. Jaksch-Wartenhorst first described it in 1923 and named it polychondropathia; in 1960 Pearson analyzed 12 cases and named it relapsing polychondritis. The cause and mechanism are unclear, but growing evidence points to autoimmune mechanisms.

Common Symptoms

Yi Yupin; ataxia; dyspnea; respiratory failure

Causes

(1) Etiology. The cause is unknown and may relate to trauma, infection, allergy, alcoholism and hydralazine hydrochloride; some relate it to mesodermal synthesis disorder or abnormal proteolytic enzymes, but years of study suggest an immune-mediated disease involving humoral and cellular immunity. (2) Mechanism. The mechanism is unclear; immune mediation may be key. 25%-30% of cases have other autoimmune diseases such as rheumatoid arthritis, polyarteritis nodosa, Sjögren syndrome, SLE, Behçet disease, Reiter syndrome, Wegener granulomatosis, ankylosing spondylitis and vasculitis. Pathology shows mononuclear infiltration, especially CD4 lymphocytes and plasma cells; serology finds anti-type-II collagen antibodies, and a few have anti-type-IX and -XI collagen antibodies; some are positive for ANA, rheumatoid factor or circulating immune complexes; immunizing rodents with type-II collagen causes auricular and polyarticular cartilage inflammation; patients show enhanced cell-mediated specific immunity to cartilage antigens; direct immunofluorescence shows immunoglobulin and complement deposition in affected cartilage; RP is associated with HLA-DR4 but not HLA class I. Buker et al. recently reported that Matrilin-1, a cartilage matrix protein specific to adult tracheal, ear and nasal cartilage, may be involved; glucocorticoids or immunosuppressants are effective. In sum, RP is an autoimmune response mainly against type-II collagen causing cartilage destruction; cartilage glycoprotein, elastin and other collagens can also trigger it; cartilage glycoprotein is widespread in sclera, iris ciliary body, trachea, optic nerve, endothelium, aortic media, heart valves, myocardial fibril membrane, glomerular basement membrane and synovium, and its antibodies can induce chondral degeneration, synovitis and perichondritis; glycoprotein also inhibits chondrocyte glycoprotein synthesis, and its role needs clarification. RP has no specific pathology; its histological feature is chondrolysis with perichondritis: initially acute and chronic inflammatory cells (mono- and polynuclear cells, fibroblasts, vascular endothelium) infiltrate the cartilage-perichondrium junction; then acid mucopolysaccharides in the matrix decrease or vanish, the matrix loosens and chondrocytes are destroyed; as it progresses, the matrix necroses, lyses and liquefies with granulation tissue; finally residual cartilage disappears, granulation fibrosis, scars contract and tissue collapses and deforms.

Treatment

Medication, supportive treatment

Prevention

Avoiding triggering immune responses is key to preventing autoimmune disease. 1. Remove or reduce risk factors, improve living environment, keep good habits, prevent infection, maintain dietary hygiene and eat a balanced diet. 2. Exercise to build resistance; avoid overfatigue and overconsumption; stop smoking and limit alcohol.

Recommended Department

It is recommended to visit the orthopedic surgery department; before the visit you may first book an appointment and connect with experts through this platform.

Warm reminder: The above information is provided for health education reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek care at a regular hospital in time.