ERN ReCONNET
Disease Info
Disease: Relapsing Polychondritis
Relapsing Polychondritis (RP)
Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent and destructive inflammatory lesions of the cartilages of the nose, ears and tracheobronchial tree. Additional clinical features include ocular inflammation, audio-vestibular impairment, vasculitis, skin involvement, valvular insufficiency, and non-erosive arthritis. The pathogenesis of the disease is unclear but several evidence support the role for auto-immune mechanisms [1].
With an incidence of about 1 case per million per year, the rarity of the disease makes it difficult to provide a standardized approach for treatment and follow-up of RP. In particular, there is no consensus agreement on the management of this disease. In most series, males and females are equally affected by RP. The peak incidence is in the fifth decade but the disease can occur in children and older adults. In about 30% of cases, another immune-mediated disease such as rheumatoid arthritis, systemic lupus or Sjögren’s syndrome is associated with RP.
The disease has a long and unpredictable course which can range from minor relapsing chondritis to life-threatening presentations. Some tools have been specifically developed to measure disease activity and damage in RP [2]. Minor forms of the disease can be treated with non-steroidal anti-inflammatory drugs or short corticosteroid courses, whereas more severe forms are usually treated with systemic corticosteroids, immunosuppressant therapy or biologics [3].
Given the chronicity of the disease a prolonged and regular follow-up by an experienced physician is recommanded to RP patients.
LIST OF USEFUL REFERENCES
1. Relapsing polychondritis: A clinical update
Longo L, Greco A, Rea A, Lo Vasco VR, De Virgilio A, De Vincentiis M.
Autoimmun Rev. 2016 Jun;15(6):539-43.
Longo L, Greco A, Rea A, Lo Vasco VR, De Virgilio A, De Vincentiis M.
Autoimmun Rev. 2016 Jun;15(6):539-43.
3. Pathogenesis of relapsing polychondritis: a 2013 update
Arnaud L, Mathian A, Haroche J, Gorochov G, Amoura Z.
Autoimmun Rev. 2014 Feb;13(2):90-5
4. Antibodies to type II collagen in relapsing polychondritis
Foidart JM, Abe S, Martin GR, Zizic TM, Barnett EV, Lawley TJ, Katz SI.
N Engl J Med. 1978 Nov 30;299(22):1203-7.
5. The occurrence of autoantibodies to matrilin 1 reflects a tissue-specific response to cartilage of the respiratory tract in patients with relapsing polychondritis
Hansson AS, Heinegård D, Piette JC, Burkhardt H, Holmdahl R.
Arthritis Rheum. 2001 Oct;44(10):2402-12.
Arnaud L, Mathian A, Haroche J, Gorochov G, Amoura Z.
Autoimmun Rev. 2014 Feb;13(2):90-5
Foidart JM, Abe S, Martin GR, Zizic TM, Barnett EV, Lawley TJ, Katz SI.
N Engl J Med. 1978 Nov 30;299(22):1203-7.
Hansson AS, Heinegård D, Piette JC, Burkhardt H, Holmdahl R.
Arthritis Rheum. 2001 Oct;44(10):2402-12.
6. Relapsing polychondritis. Survival and predictive role of early disease manifestations
Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM.
Ann Intern Med. 1986 Jan;104(1):74-8.
7. Relapsing polychondritis – report of ten cases
Damiani JM, Levine HL.
Laryngoscope. 1979 Jun;89(6 Pt 1):929-46.
8. Utility of fluorodeoxyglucose positron emission tomography/computed tomography for early diagnosis and evaluation of disease activity of relapsing polychondritis: a case series and literature review
Yamashita H, Takahashi H, Kubota K, Ueda Y, Ozaki T, Yorifuji H, Bannai E, Minamimoto R, Morooka M, Miyata Y, Okasaki M, Takahashi Y, Kaneko H, Kano T, Mimori A.
Rheumatology (Oxford). 2014 Aug;53(8):1482-90.
9. Relapsing Polychondritis Can Be Characterized by Three Different Clinical Phenotypes: Analysis of a Recent Series of 142 Patients
Dion J, Costedoat-Chalumeau N, Sène D, Cohen-Bittan J, Leroux G, Dion C, Francès C, Piette JC.
Arthritis Rheumatol. 2016 Dec;68(12):2992-3001. doi: 10.1002/art.39790.
10. Aortic involvement in relapsing polychondritis
Le Besnerais M, Arnaud L, Boutémy J, Bienvenu B, Lévesque H, Amoura Z, Marie I.
Joint Bone Spine. 2018 May;85(3):345-351.
Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM.
Ann Intern Med. 1986 Jan;104(1):74-8.
Damiani JM, Levine HL.
Laryngoscope. 1979 Jun;89(6 Pt 1):929-46.
Yamashita H, Takahashi H, Kubota K, Ueda Y, Ozaki T, Yorifuji H, Bannai E, Minamimoto R, Morooka M, Miyata Y, Okasaki M, Takahashi Y, Kaneko H, Kano T, Mimori A.
Rheumatology (Oxford). 2014 Aug;53(8):1482-90.
Dion J, Costedoat-Chalumeau N, Sène D, Cohen-Bittan J, Leroux G, Dion C, Francès C, Piette JC.
Arthritis Rheumatol. 2016 Dec;68(12):2992-3001. doi: 10.1002/art.39790.
Le Besnerais M, Arnaud L, Boutémy J, Bienvenu B, Lévesque H, Amoura Z, Marie I.
Joint Bone Spine. 2018 May;85(3):345-351.
Treatment of relapsing polychondritis: a systematic review
Petitdemange A, Sztejkowski C, Damian L, Martin T, Mouthon L, Amoura Z, Cutolo M, Burmester GR, Fonseca JE, Rednic S, Arnaud L.
Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):81-85.
The relapsing polychondritis damage index (RPDAM): Development of a disease-specific damage score for relapsing polychondritis
Mertz P, Belot A, Cervera R, Chuah TY, Dagna L, Damian L, Danda D, D’cruz D, Espinosa G, Frances C, Jayne D, Ooi KK, Kucharz EJ, Lebovics R, Marie I, Moulis G, Peng S, Sharma A, Suzuki N, Tanaka T, Van Vollenhoven R, Sibilia J, Gottenberg JE, Chasset F, Arnaud L.
Joint Bone Spine. 2019 May;86(3):363-368.
Efficacy and safety of biologics in relapsing polychondritis: a French national multicentre study
Moulis G, Pugnet G, Costedoat-Chalumeau N, Mathian A, Leroux G, Boutémy J, Espitia O, Bouillet L, Berthier S, Gaultier JB, Jeandel PY, Konaté A, Mékinian A, Solau-Gervais E, Terrier B, Wendling D, Andry F, Garnier C, Cathébras P, Arnaud L, Palmaro A, Cacoub P, Amoura Z, Piette JC, Arlet P, Lapeyre-Mestre M, Sailler L.
Ann Rheum Dis. 2018 Mar 13
The Relapsing Polychondritis Disease Activity Index: development of a disease activity score for relapsing polychondritis
Arnaud L, Devilliers H, Peng SL, Mathian A, Costedoat-Chalumeau N, Buckner J, Dagna L, Michet C, Sharma A, Cervera R, Haroche J, Papo T, D’Cruz D, Arlet P, Zwerina J, Belot A, Suzuki N, Harle JR, Moots R, Jayne D, Hachulla E, Marie I, Tanaka T, Lebovics R, Scott D, Kucharz EJ, Birchall M, Kong KO, Gorochov G, Amoura Z; RPDAI study group.
Autoimmun Rev. 2012 Dec;12(2):204-9
Treatment of relapsing polychondritis with rituximab: a retrospective study of nine patients
Leroux G, Costedoat-Chalumeau N, Brihaye B, Cohen-Bittan J, Amoura Z, Haroche J, Limal N, Bletry O, Piette JC.
Arthritis Rheum. 2009 May 15;61(5):577-82
Disease activity and damage tools for patient follow-up
Petitdemange A, Sztejkowski C, Damian L, Martin T, Mouthon L, Amoura Z, Cutolo M, Burmester GR, Fonseca JE, Rednic S, Arnaud L.
Clin Exp Rheumatol. 2022 May;40 Suppl 134(5):81-85.
Mertz P, Belot A, Cervera R, Chuah TY, Dagna L, Damian L, Danda D, D’cruz D, Espinosa G, Frances C, Jayne D, Ooi KK, Kucharz EJ, Lebovics R, Marie I, Moulis G, Peng S, Sharma A, Suzuki N, Tanaka T, Van Vollenhoven R, Sibilia J, Gottenberg JE, Chasset F, Arnaud L.
Joint Bone Spine. 2019 May;86(3):363-368.
Moulis G, Pugnet G, Costedoat-Chalumeau N, Mathian A, Leroux G, Boutémy J, Espitia O, Bouillet L, Berthier S, Gaultier JB, Jeandel PY, Konaté A, Mékinian A, Solau-Gervais E, Terrier B, Wendling D, Andry F, Garnier C, Cathébras P, Arnaud L, Palmaro A, Cacoub P, Amoura Z, Piette JC, Arlet P, Lapeyre-Mestre M, Sailler L.
Ann Rheum Dis. 2018 Mar 13
Arnaud L, Devilliers H, Peng SL, Mathian A, Costedoat-Chalumeau N, Buckner J, Dagna L, Michet C, Sharma A, Cervera R, Haroche J, Papo T, D’Cruz D, Arlet P, Zwerina J, Belot A, Suzuki N, Harle JR, Moots R, Jayne D, Hachulla E, Marie I, Tanaka T, Lebovics R, Scott D, Kucharz EJ, Birchall M, Kong KO, Gorochov G, Amoura Z; RPDAI study group.
Autoimmun Rev. 2012 Dec;12(2):204-9
Leroux G, Costedoat-Chalumeau N, Brihaye B, Cohen-Bittan J, Amoura Z, Haroche J, Limal N, Bletry O, Piette JC.
Arthritis Rheum. 2009 May 15;61(5):577-82
Disease activity and damage tools for patient follow-up
