Relapsing polychondritis
Authors:
Jozef Rovenský 1; Marie Sedláčková 2
Authors place of work:
Národný ústav reumatických chorôb, Piešťany
1; Revmatologické a rehabilitační oddělení Fakultní Thomayerová nemocnice, Praha
2
Published in the journal:
Čas. Lék. čes. 2012; 151: 64-68
Category:
Review Article
Summary
Relapsing polychondritis (RP) is an unusually rare disease involving multiple organs. It has an episodic course, occasionally also progressing. Typically, inflammation of cartilagenous tissues and tissues rich in glycosaminoglycans is present. Clinical symptoms are concentrated in auricula, nose, larynx, upper respiratory tract, joints, heart, blood vessels, inner ear, cornea and sclera.
Manifestations include:
- Chondritis of auricular, nasal, laryngotracheal, costal and joint cartilages
- Inflammation of the eyes and inner ear
- Collapse of laryngotracheal structures and structures in the subglottic area resulting in increased susceptibility to upper respiratory tract infections
- Diversity of clinical manifestations, of the disease course and also of the treatment response
Concurrent systemic vasculitis or glomerulonephritis may contribute to higher morbidity and premature mortality.
In about 30 % of cases the RP is secondary, accompanied by other systemic connective tissue disorders as RA, SLE, Sjögren‘s syndrome, thyroiditis, ulcerative colitis, psoriasis and Behçet‘s syndrome.
Diagnosis is based on 1986 diagnostic criteria from Minnesota and RP has to be suspected when the inflammatory bouts involve at least two of the typical sites – auricular, nasal, laryngotracheal or one of the typical sites and two other – ocular, statoacoustic disturbances (hearing loss and/or vertigo) and arthritis.
In the treatment are apart from corticoids and nonsteroidal anti-inflammatory drugs also corticoids combined with immunosupressive therapy (cyclophosphamide, azathioprine, chlorambucil, cyclosporine) used. More recently, also biologic therapy is used in RP (infliximab, adalimumab, ethanercept, tocilizumab, rituximab). It is necessary to underscore that biologic therapy for RP is only a research modality used in very severe refractory forms of RP. Preliminary results suggest that biologic therapy will have its place in severe refractory relapsing forms of RP.
Key words:
relapsing polychondritis, inflammatory damage of cartilage, primary and secondary form of RP, treatment of RP with classical immunosupressive corticoids, biologic therapy
Zdroje
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