Rezidivierende Polychondritis: ein Chamäleon unter den seltenen Erkrankungen

Abstract

Relapsing polychondritis (RPC) is a rare disease with recurrent episodes of inflammation of cartilage tissue leading to fibrosis and organ damage. Despite unknown etiology, there is some evidence of a genetic predisposition. The clinical presentation is heterogeneous and an association with other autoimmune disorders such as rheumatoid arthritis or different forms of vasculitis has been described. All organ systems containing cartilage can be affected, such as ear, nose, joints, trachea, aorta, and coronary arteries. Given the broad spectrum of potential manifestations, a variety of medical specialists may be involved in the management of RPC patients. As establishing the diagnosis of RPC may be difficult, an interdisciplinary approach may be preferable. Treatment options include glucocorticoids, dapsone, disease-modifying antirheumatic drugs, and biologics. Prognosis is as heterogeneous as the clinical picture, depending on the severity of organ damage. In this paper we give an overview of the current knowledge with regard to pathogenesis, clinical picture, diagnosis, and therapy of RPC.

Replasing Polychondritis and Geriatrics: Report of Two Cases

“…The diagnosis of RP rests on clinical grounds and is frequently delayed [6]. Michet et al developed criteria for diagnosing RP [5][6][7][10][11][12]. An advantage of their criteria is that a biopsy does not need to be obtained routinely [10]; Al though not validated, the criteria of Michet et al are useful in clinical practice.…”

“…Criteria for diagnosing relapsing polychondritis [6,7,10,12]. Two major criteria or one major criteria+two minor criteria are needed to classify the patient as having relapsing polychondritis.…”

Relapsing Polychondritis Misdiagnosed and Treated as a ''Soft Tissue Infection'' and a Brief Review of Diagnostic Clinical Clues

“…inflammatory bowel diseases, systemic vasculitides (ANCA-associated, polyarteritis nodosa, Behcet's disease, necrotizing vasculitis, etc) and an increased prevalance of myelodisplastic syndromes are reported. [4][5][6] RPC is also reported to manifest after an intravenous injection of a mixture, which consisted of hydrochloric acid, carburetor fluid, tap water, and internal matrix of a nasal inhaler. 7 Diversity of the clinical symptoms, associated genes and coexistent diseases indicate the possibility of RPC being a syndrome rather than a single, uniform disease.…”

Impact of an Exercise Training Program in a Patient with Relapsing Polychondritis: A Case Report

“…Patients with RP have recurrent inflammation of auricular pinna, nasal cartilage, peripheral joints and vertebral disks [2] [3]. Moreover, these patients Open Journal of Rheumatology and Autoimmune Diseases may exhibit clinical manifestations, such as auricular chondritis, non-erosive polyarthritis, nasal cartilage chondritis, inflammation of ocular structures, respiratory tract chondritis, as well as vestibular and cochlear damage [2] [3] [4].…”

“…Structural deformities can lead to life-threatening cardiopulmonary manifestations, such as airway collapse and valvarcardiac regurgitation [4] [5]. Generally, the primary cause of death is respiratory failure due to RP, followed by lung infections and cardiovascular events [1]…”