Relapsing polychondritis
Authors:
Jozef Rovenský 1; Marie Sedláčková 2
Authors‘ workplace:
Národný ústav reumatických chorôb, Piešťany
1; Revmatologické a rehabilitační oddělení Fakultní Thomayerová nemocnice, Praha
2
Published in:
Čas. Lék. čes. 2012; 151: 64-68
Category:
Review Article
Overview
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
Sources
1. Jaksch-Wartenhorst R. Polychondropathia. Wiener Archiv für Inn Med 1923; 6: 93–94.
2. Ebringer R, et al. Autoantibodies to cartilage and type II collagen in relapsing polychondritis and other rheumatic diseases. Ann Rheum Dis 1981; 40: 473–479.
3. Arundell FW, Haserick JR. Familial chronic atrophic polychondritis. Arch Dermatol 1960; 82: 353–365.
4. Tomík F, et al. Recidivujúca polychondritída. Čsl Patologie 1977; 13: 91–94.
5. Neilly JB, et al. Progressive tracheobronchial polychondritis: need of early diagnosis. Thorax 1985; 40: 78–79.
6. Bellamy N, Dewar CL. Relapsing polychondritis in pregnancy. J Rheumatol 1990; 17: 1525–1526.
7. Sallam A, et al. Keratouveitis as a first presentation of relapsing polychondritis. Hindawi Publishing Corporations 2010. Case report In Medicine, Article ID 176514, 2 pages, doi: 10.1155/2010/176514
8. Starr JC, et al. Relapsing polychondritis following alopecia areata. Hindawi Publishing Corporations 2010. Case report In Medicine, Article ID 623158, 3 pages, doi: 10.1155/2010/623158
9. Erten-Lyons D, et al. Relapsing polychondritis: an uncommon cause of dementia. BMJ Publishing Group 2009. Case report bcr08.2008.0740, Published online doi: 10.1136/bcr.08. 2008.0740
10. Bochtler T, et al. Chronic lymphocytic leukaemia and concominant relapsing polychondritis: a report on one treatment for the combined manifestation of two diseases. Rheumatology 2005; 44: 1199.
11. Kim MK, et al. A case of polychondritis in a patient with Behćetęs disease. Korean J Intern Med 2005; 20: 339–342.
12. Duda J, Botka M. Neobvyklý prípad relabujúcej polychondritídy. Rheumatologia 2001; 15: 136.
13. McAdam L, et al. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976; 55: 193–215.
14. Damiani JM, Levine HL. Relapsing polychondritis. Laryngoscope 1979; 89: 929–946.
15. Van der Lubbe PA, et al. Anti-CD4 monoclonal antibody for relapsing polychondritis. Lancet 1991; 337: 1349.
16. Handler RP. Leflunomide for relapsing polychondritis: successful longterm teatment. J Rheumatol 2006; 33: 1916.
17. Koenig AS, Abruzzo JL. Leflunomide induced fevers, thrombocytosis, and leukocytosis in a patient with relapsing polychondritis. J Rheumatol 2002; 29: 192–194.
18. Ehresmann GR. Infliximab in the treatment of polychondritis. ACR Poster Session A. Pathogenesis and treatment of rare rheumatic diseases II. 2002.
19. de Baros AP, et al. Infliximab in relapsing polychondritis. Rev Bras Reumatol 2010; 50: 211–1216.
20. Carter JD. Treatment of relapsing polychondritis with a TNF antagonist, J Rheumatol 2005; 32: 1413.
21. Seymour MW, et al. Prolonged response to anti-tumour necrosis factor treatment with adalimumab (Humira) in relapsing polychondritis complicated by aortitis. Rheumatology 2007; 46: 1738–1739.
22. Vounotrypidis P, et al. Refractory relapsing polychondritis: rapid and sustained response in the treatment with an IL-L receptor antagonist (anakinra). Rheumatology 2006; 45: 491–492.
23. Kawai M, et al. Sustained response to tocilizumab, anti-interleukine-6 receptor antibody, in two patients with refractory relapsing polychondritis. Rheumatology 2009; 48: 318–319.
24. Lipnick RL, Fink ChW. Acute airway obstruction in relapsing polychondritis: Treatment with pulse methylprednisolone. J Rheumatol 1991; 18: 98–99.
Labels
Addictology Allergology and clinical immunology Anaesthesiology, Resuscitation and Inten Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric dermatology & STDs Paediatric gastroenterology Paediatric gynaecology Paediatric surgery Paediatric cardiology Paediatric nephrology Paediatric neurology Paediatric clinical oncology Paediatric ENT Paediatric pneumology Paediatric psychiatry Paediatric radiology Paediatric rheumatology Paediatric urologist Diabetology Endocrinology Pharmacy Clinical pharmacology Physiotherapist, university degree Gastroenterology and hepatology Medical genetics Geriatrics Gynaecology and obstetrics Haematology Hygiene and epidemiology Hyperbaric medicine Vascular surgery Chest surgery Plastic surgery Surgery Medical virology Intensive Care Medicine Cardiac surgery Cardiology Clinical speech therapy Clinical microbiology Nephrology Neonatology Neurosurgery Neurology Nuclear medicine Nutritive therapist Obesitology Ophthalmology Clinical oncology Orthodontics Orthopaedics ENT (Otorhinolaryngology) Anatomical pathology Paediatrics Pneumology and ftiseology Burns medicine Medical assessment General practitioner for children and adolescents Orthopaedic prosthetics Clinical psychology Radiodiagnostics Radiotherapy Rehabilitation Reproduction medicine Rheumatology Nurse Sexuology Forensic medical examiner Dental medicine Sports medicine Toxicology Traumatology Trauma surgery Urology Laboratory Home nurse Phoniatrics Pain management Health Care Medical studentArticle was published in
Journal of Czech Physicians
Most read in this issue
- Prevalence of thrombophilic mutations of FV Leiden, prothrombin G20210A and PAI-1 4G/5G and their combinations in a group of 1,450 healthy middle-aged individuals in the Prague and Central Bohemian regions (results of FRET real-time PCR assay)
- Relapsing polychondritis
- Male hypogonadism and civilization diseases
- Morphology and etiopathogenesis of the abdominal aortic aneurysm