Skip to main content

Tipp

Weitere Artikel dieser Ausgabe durch Wischen aufrufen

Erschienen in: Wiener Medizinische Wochenschrift 9-10/2017

01.06.2017 | main topic

Relapsing polychondritis: a chameleon among orphan diseases

verfasst von: MD Sabine Schumacher, MD, MBA Herwig Pieringer

Erschienen in: Wiener Medizinische Wochenschrift | Ausgabe 9-10/2017

Einloggen, um Zugang zu erhalten
share
TEILEN

Summary

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.
Literatur
1.
Zurück zum Zitat Zeuner M, Straub RH, Rauh G, Albert ED, Schölmerich J, Lang B. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997;24(1):96–101. PubMed Zeuner M, Straub RH, Rauh G, Albert ED, Schölmerich J, Lang B. Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients. J Rheumatol. 1997;24(1):96–101. PubMed
2.
Zurück zum Zitat Hue-Lemoine S, Caillat-Zucman S, Amoura Z, et al. Arthritis Rheum. 1999;42:261. Hue-Lemoine S, Caillat-Zucman S, Amoura Z, et al. Arthritis Rheum. 1999;42:261.
3.
Zurück zum Zitat Berger R. Polychondritis resulting from intravenous substance abuse. Am J Med. 1988;85(3):415–7. CrossRefPubMed Berger R. Polychondritis resulting from intravenous substance abuse. Am J Med. 1988;85(3):415–7. CrossRefPubMed
5.
Zurück zum Zitat Hebbar M, Hebbar-Savéan K, Fenaux P. Systemic diseases in myelodysplastic syndromes. Soc Natl Fr Med Interne. 1995;16(12):897–904. Hebbar M, Hebbar-Savéan K, Fenaux P. Systemic diseases in myelodysplastic syndromes. Soc Natl Fr Med Interne. 1995;16(12):897–904.
6.
Zurück zum Zitat McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore). 1976;55(3):193–215. CrossRef McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore). 1976;55(3):193–215. CrossRef
7.
9.
Zurück zum Zitat Stabler T, Piette JC, Chevalier X, Marini-Portugal A, Kraus VB. Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthritis Rheum. 2004;50(11):3663–7. doi: 10.​1002/​art.​20613. CrossRefPubMed Stabler T, Piette JC, Chevalier X, Marini-Portugal A, Kraus VB. Serum cytokine profiles in relapsing polychondritis suggest monocyte/macrophage activation. Arthritis Rheum. 2004;50(11):3663–7. doi: 10.​1002/​art.​20613. CrossRefPubMed
10.
Zurück zum Zitat Buckner JH, Van Landeghen M, Kwok WW, Tsarknaridis L. Identification of type II collagen peptide 261–273-specific T cell clones in a patient with relapsing polychondritis. Arthritis Rheum. 2002;46(1):238–44. doi:10.1002/1529-0131(200201)46:1〈238::AID-ART10030〉3.0.CO;2-M. CrossRefPubMed Buckner JH, Van Landeghen M, Kwok WW, Tsarknaridis L. Identification of type II collagen peptide 261–273-specific T cell clones in a patient with relapsing polychondritis. Arthritis Rheum. 2002;46(1):238–44. doi:10.1002/1529-0131(200201)46:1〈238::AID-ART10030〉3.0.CO;2-M. CrossRefPubMed
11.
Zurück zum Zitat Riccieri V, Spadaro A, Taccari E, Zoppini A. A case of relapsing polychondritis: pathogenetic considerations. Clin Exp Rheumatol. 1988;6(1):95–6. PubMed Riccieri V, Spadaro A, Taccari E, Zoppini A. A case of relapsing polychondritis: pathogenetic considerations. Clin Exp Rheumatol. 1988;6(1):95–6. PubMed
13.
Zurück zum Zitat Coppola M, Yealy DM. Relapsing polychondritis: an unusual cause of painful auricular swelling. Ann Emerg Med. 1992;21(1):81–5. CrossRefPubMed Coppola M, Yealy DM. Relapsing polychondritis: an unusual cause of painful auricular swelling. Ann Emerg Med. 1992;21(1):81–5. CrossRefPubMed
16.
Zurück zum Zitat Isaak BL, Liesegang TJ, Michet CJJ. Ocular and systemic findings in relapsing polychondritis. Ophthalmology. 1986;93(5):681–9. CrossRefPubMed Isaak BL, Liesegang TJ, Michet CJJ. Ocular and systemic findings in relapsing polychondritis. Ophthalmology. 1986;93(5):681–9. CrossRefPubMed
23.
Zurück zum Zitat Balsa A, Espinosa A, Cuesta M, MacLeod TI, Gijon-Banos J, Maddison PJ. Joint symptoms in relapsing polychondritis. Clin Exp Rheumatol. 1995;13(4):425–30. PubMed Balsa A, Espinosa A, Cuesta M, MacLeod TI, Gijon-Banos J, Maddison PJ. Joint symptoms in relapsing polychondritis. Clin Exp Rheumatol. 1995;13(4):425–30. PubMed
24.
Zurück zum Zitat Lang-Lazdunski L, Hvass U, Paillole C, Pansard Y, Langlois J. Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis. 1995;4(3):227–35. PubMed Lang-Lazdunski L, Hvass U, Paillole C, Pansard Y, Langlois J. Cardiac valve replacement in relapsing polychondritis. A review. J Heart Valve Dis. 1995;4(3):227–35. PubMed
26.
Zurück zum Zitat Sugrue G, Durcan L, Bell L, Bolster F, Donnelly S, Kavanagh E. Unsuspected cardiovascular involvement in relapsing polychondritis: a case of aortitis with critical coronary artery stenosis secondary to relapsing polychondritis. Circ Cardiovasc Imaging. 2014;7(2):409–11. doi: 10.​1161/​CIRCIMAGING.​113.​001290. CrossRefPubMed Sugrue G, Durcan L, Bell L, Bolster F, Donnelly S, Kavanagh E. Unsuspected cardiovascular involvement in relapsing polychondritis: a case of aortitis with critical coronary artery stenosis secondary to relapsing polychondritis. Circ Cardiovasc Imaging. 2014;7(2):409–11. doi: 10.​1161/​CIRCIMAGING.​113.​001290. CrossRefPubMed
27.
Zurück zum Zitat Buckley LM, Ades PA. Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis. Arthritis Rheum. 1992;35(7):812–4. CrossRefPubMed Buckley LM, Ades PA. Progressive aortic valve inflammation occurring despite apparent remission of relapsing polychondritis. Arthritis Rheum. 1992;35(7):812–4. CrossRefPubMed
28.
Zurück zum Zitat Chang-Miller A, Okamura M, Torres VE, et al. Renal involvement in relapsing polychondritis. Medicine (Baltimore). 1987;66(3):202–17. CrossRef Chang-Miller A, Okamura M, Torres VE, et al. Renal involvement in relapsing polychondritis. Medicine (Baltimore). 1987;66(3):202–17. CrossRef
29.
Zurück zum Zitat Sundaram MB, Rajput AH. Nervous system complications of relapsing polychondritis. Neurology. 1983;33(4):513–5. CrossRefPubMed Sundaram MB, Rajput AH. Nervous system complications of relapsing polychondritis. Neurology. 1983;33(4):513–5. CrossRefPubMed
30.
Zurück zum Zitat Hanslik T, Wechsler B, Piette JC, Vidailhet M, Robin PM, Godeau P. Central nervous system involvement in relapsing polychondritis. Clin Exp Rheumatol. 1994;12(5):539–41. PubMed Hanslik T, Wechsler B, Piette JC, Vidailhet M, Robin PM, Godeau P. Central nervous system involvement in relapsing polychondritis. Clin Exp Rheumatol. 1994;12(5):539–41. PubMed
33.
34.
Zurück zum Zitat Firestein GS, Gruber HE, Weisman MH, Zvaifler NJ, Barber J, O’Duffy JD. Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behçet’s disease. Am J Med. 1985;79(1):65–72. CrossRefPubMed Firestein GS, Gruber HE, Weisman MH, Zvaifler NJ, Barber J, O’Duffy JD. Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behçet’s disease. Am J Med. 1985;79(1):65–72. CrossRefPubMed
35.
Zurück zum Zitat Orme RL, Nordlund JJ, Barich L, Brown T. The MAGIC syndrome (mouth and genital ulcers with inflamed cartilage). Arch Dermatol. 1990;126(7):940–4. CrossRefPubMed Orme RL, Nordlund JJ, Barich L, Brown T. The MAGIC syndrome (mouth and genital ulcers with inflamed cartilage). Arch Dermatol. 1990;126(7):940–4. CrossRefPubMed
36.
Zurück zum Zitat Michet CJ, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med. 1986;104(1):74–8. CrossRefPubMed Michet CJ, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med. 1986;104(1):74–8. CrossRefPubMed
38.
Zurück zum Zitat Iribe H, Kabashima H, Ishii Y, Koga T. Epitope specificity of antibody response against human type II collagen in the mouse susceptible to collagen-induced arthritis and patients with rheumatoid arthritis. Clin Exp Immunol. 1988;73(3):443–8. PubMedPubMedCentral Iribe H, Kabashima H, Ishii Y, Koga T. Epitope specificity of antibody response against human type II collagen in the mouse susceptible to collagen-induced arthritis and patients with rheumatoid arthritis. Clin Exp Immunol. 1988;73(3):443–8. PubMedPubMedCentral
39.
43.
Zurück zum Zitat Thaiss WM, Nikolaou K, Spengler W, et al. Imaging diagnosis in relapsing polychondritis and correlation with clinical and serological data. Skeletal Radiol. 2015; doi: 10.​1007/​s00256-015-2270-x. Thaiss WM, Nikolaou K, Spengler W, et al. Imaging diagnosis in relapsing polychondritis and correlation with clinical and serological data. Skeletal Radiol. 2015; doi: 10.​1007/​s00256-015-2270-x.
44.
Zurück zum Zitat Deng H, Chen P, Wang L, Li X, Yi J. Relapsing polychondritis on PET/CT. Clin Nucl Med. 2012;37(7):712–5. CrossRefPubMed Deng H, Chen P, Wang L, Li X, Yi J. Relapsing polychondritis on PET/CT. Clin Nucl Med. 2012;37(7):712–5. CrossRefPubMed
46.
Zurück zum Zitat Iida M, Orime Y, Umeda T, Ishii Y, Shiono M. Emergency repair of acute aortic dissection in a patient with relapsing polychondritis. Ann Thorac Cardiovasc Surg. 2013;19(2):158–61. CrossRefPubMed Iida M, Orime Y, Umeda T, Ishii Y, Shiono M. Emergency repair of acute aortic dissection in a patient with relapsing polychondritis. Ann Thorac Cardiovasc Surg. 2013;19(2):158–61. CrossRefPubMed
47.
Zurück zum Zitat Damiani JM, Levine HL. Relapsing polychondritis – report of ten cases. Laryngoscope. 1979;89(6 Pt 1):929–46. PubMed Damiani JM, Levine HL. Relapsing polychondritis – report of ten cases. Laryngoscope. 1979;89(6 Pt 1):929–46. PubMed
48.
Zurück zum Zitat Askari AD. Colchicine for treatment of relapsing polychondritis. J Am Acad Dermatol. 1984;10(3):507–10. CrossRefPubMed Askari AD. Colchicine for treatment of relapsing polychondritis. J Am Acad Dermatol. 1984;10(3):507–10. CrossRefPubMed
49.
Zurück zum Zitat Mark KA, Franks AG. Colchicine and indomethacin for the treatment of relapsing polychondritis. J Am Acad Dermatol. 2002;46(2 Suppl):S22–S24. CrossRefPubMed Mark KA, Franks AG. Colchicine and indomethacin for the treatment of relapsing polychondritis. J Am Acad Dermatol. 2002;46(2 Suppl):S22–S24. CrossRefPubMed
50.
Zurück zum Zitat Ruhlen JL, Huston KA, Wood WG. Relapsing polychondritis with glomerulonephritis. Improvement with prednisone and cyclophosphamide. JAMA. 1981;245(8):847–8. CrossRefPubMed Ruhlen JL, Huston KA, Wood WG. Relapsing polychondritis with glomerulonephritis. Improvement with prednisone and cyclophosphamide. JAMA. 1981;245(8):847–8. CrossRefPubMed
51.
Zurück zum Zitat Hughes RA, Berry CL, Seifert M, Lessof MH. Relapsing polychondritis. Three cases with a clinico-pathological study and literature review. Q J Med. 1972;41(163):363–80. PubMed Hughes RA, Berry CL, Seifert M, Lessof MH. Relapsing polychondritis. Three cases with a clinico-pathological study and literature review. Q J Med. 1972;41(163):363–80. PubMed
52.
Zurück zum Zitat Priori R, Paroli MP, Luan FL, Abdulaziz M, Pivetti Pezzi P, Valesini G. Cyclosporin A in the treatment of relapsing polychondritis with severe recurrent eye involvement. Br J Rheumatol. 1993;32(4):352. CrossRefPubMed Priori R, Paroli MP, Luan FL, Abdulaziz M, Pivetti Pezzi P, Valesini G. Cyclosporin A in the treatment of relapsing polychondritis with severe recurrent eye involvement. Br J Rheumatol. 1993;32(4):352. CrossRefPubMed
53.
Zurück zum Zitat Park J, Gowin KM, Schumacher HR. Steroid sparing effect of methotrexate in relapsing polychondritis. J Rheumatol. 1996;23(5):937–8. PubMed Park J, Gowin KM, Schumacher HR. Steroid sparing effect of methotrexate in relapsing polychondritis. J Rheumatol. 1996;23(5):937–8. PubMed
54.
Zurück zum Zitat Handler RP. Leflunomide for relapsing polychondritis: successful longterm treatment. J Rheumatol. 2006;33(9):1916, author reply 1916–1917. PubMed Handler RP. Leflunomide for relapsing polychondritis: successful longterm treatment. J Rheumatol. 2006;33(9):1916, author reply 1916–1917. PubMed
55.
Zurück zum Zitat Richez C, Dumoulin C, Coutouly X, Schaeverbeke T. Successful treatment of relapsing polychondritis with infliximab. Clin Exp Rheumatol. 2004;22(5):629–31. PubMed Richez C, Dumoulin C, Coutouly X, Schaeverbeke T. Successful treatment of relapsing polychondritis with infliximab. Clin Exp Rheumatol. 2004;22(5):629–31. PubMed
57.
Zurück zum Zitat Carter JD. Treatment of relapsing polychondritis with a TNF antagonist. J Rheumatol. 2005;32(7):1413. PubMed Carter JD. Treatment of relapsing polychondritis with a TNF antagonist. J Rheumatol. 2005;32(7):1413. PubMed
61.
Zurück zum Zitat Seymour MW, Home DM, Williams RO, Allard SA. Prolonged response to anti-tumour necrosis factor treatment with adalimumab (Humira) in relapsing polychondritis complicated by aortitis. Rheumatology (Oxford). 2007;46(11):1738–9. doi: 10.​1093/​rheumatology/​kem229. CrossRef Seymour MW, Home DM, Williams RO, Allard SA. Prolonged response to anti-tumour necrosis factor treatment with adalimumab (Humira) in relapsing polychondritis complicated by aortitis. Rheumatology (Oxford). 2007;46(11):1738–9. doi: 10.​1093/​rheumatology/​kem229. CrossRef
63.
Zurück zum Zitat Hernández MV, Ruiz-Esquide V, Gómez-Caballero ME, Gómez-Puerta JA, Cañete JD, Sanmartí R. Relapsing polychondritis: a new adverse event secondary to the use of tumour necrosis factor antagonists? Rheumatology (Oxford). 2011;50(8):1523–5. doi: 10.​1093/​rheumatology/​ker144. CrossRef Hernández MV, Ruiz-Esquide V, Gómez-Caballero ME, Gómez-Puerta JA, Cañete JD, Sanmartí R. Relapsing polychondritis: a new adverse event secondary to the use of tumour necrosis factor antagonists? Rheumatology (Oxford). 2011;50(8):1523–5. doi: 10.​1093/​rheumatology/​ker144. CrossRef
67.
Zurück zum Zitat Vounotrypidis P, Sakellariou GT, Zisopoulos D, Berberidis C. Refractory relapsing polychondritis: rapid and sustained response in the treatment with an IL-1 receptor antagonist (anakinra). Rheumatology (Oxford). 2006;45(4):491–2. doi: 10.​1093/​rheumatology/​kel041. CrossRef Vounotrypidis P, Sakellariou GT, Zisopoulos D, Berberidis C. Refractory relapsing polychondritis: rapid and sustained response in the treatment with an IL-1 receptor antagonist (anakinra). Rheumatology (Oxford). 2006;45(4):491–2. doi: 10.​1093/​rheumatology/​kel041. CrossRef
68.
Zurück zum Zitat Narshi CB, Allard SA. Sustained response to tocilizumab, anti-IL-6 antibody, following anti-TNF-α failure in a patient with relapsing polychondritis complicated by aortitis. Rheumatology (Oxford). 2012;51(5):952–3. doi: 10.​1093/​rheumatology/​ker451. CrossRef Narshi CB, Allard SA. Sustained response to tocilizumab, anti-IL-6 antibody, following anti-TNF-α failure in a patient with relapsing polychondritis complicated by aortitis. Rheumatology (Oxford). 2012;51(5):952–3. doi: 10.​1093/​rheumatology/​ker451. CrossRef
69.
Zurück zum Zitat Stael R, Smith V, Wittoek R, Creytens D, Mielants H. Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review. Clin Rheumatol. 2015;34(1):189–93. doi: 10.​1007/​s10067-014-2670-7. CrossRefPubMed Stael R, Smith V, Wittoek R, Creytens D, Mielants H. Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review. Clin Rheumatol. 2015;34(1):189–93. doi: 10.​1007/​s10067-014-2670-7. CrossRefPubMed
71.
72.
Zurück zum Zitat Moulis G, Sailler L, Pugnet G, Astudillo L, Arlet P. Biologics in relapsing polychondritis: a case series. Clin Exp Rheumatol. 2010;31(6):937–9. Moulis G, Sailler L, Pugnet G, Astudillo L, Arlet P. Biologics in relapsing polychondritis: a case series. Clin Exp Rheumatol. 2010;31(6):937–9.
74.
Zurück zum Zitat Spraggs PD, Tostevin PM, Howard DJ. Management of laryngotracheobronchial sequelae and complications of relapsing polychondritis. Laryngoscope. 1997;107(7):936–41. CrossRefPubMed Spraggs PD, Tostevin PM, Howard DJ. Management of laryngotracheobronchial sequelae and complications of relapsing polychondritis. Laryngoscope. 1997;107(7):936–41. CrossRefPubMed
Metadaten
Titel
Relapsing polychondritis: a chameleon among orphan diseases
verfasst von
MD Sabine Schumacher
MD, MBA Herwig Pieringer
Publikationsdatum
01.06.2017
Verlag
Springer Vienna
Erschienen in
Wiener Medizinische Wochenschrift / Ausgabe 9-10/2017
Print ISSN: 0043-5341
Elektronische ISSN: 1563-258X
DOI
https://doi.org/10.1007/s10354-017-0559-1

Weitere Artikel der Ausgabe 9-10/2017

Wiener Medizinische Wochenschrift 9-10/2017 Zur Ausgabe