Relapsing Polychondritis

Background

This is an autoimmune disease which presents in adulthood with deformities of the ears, eyes, nose, larynx, joints, and various internal organs. It is caused by antibodies to type II collagen, which is found in cartilage and the eyes.

Under the microscope, there is loss of the usual basophilic staining of cartilage, replaced with eosinophilia. Inflamatory cells range from neutrophils early in the course of the disease to plasma cells. With time, fibroblasts form a scar. In late stages, cystic degenerative spaces form within the cartilage.

OUTLINE

Disease Associations

Pathogenesis

Laboratory/Radiologic/Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Prognosis

Treatment

Commonly Used Terms

Internet Links

DISEASE ASSOCIATIONS CHARACTERIZATION

Myelodysplastic syndrome associated with relapsing polychondritis: unusual transformation from refractory anemia to chronic myelomonocytic leukemia.

Shirota T, Hayashi O, Uchida H, Tonozuka N, Sakai N, Itoh H.

Department of Internal Medicine, Tokyo Medical College Hospital, Japan.

Ann Hematol 1993 Jul;67(1):45-7 Abstract quote

The authors report an unusual case of myelodysplastic syndrome (MDS) associated with relapsing polychondritis (RP), which developed at almost the same time as MDS.

The initial diagnosis was MDS, refractory anemia (RA) subtype, according to the FAB classification. Symptoms of RP were apparently controlled by oral administration of prednisolone (PSL), although MDS was not. Within 1 month after the diagnosis, monocytosis and thrombocytopenia without excess of blasts became prominent and transformation from RA to chronic myelomonocytic leukemia (CMML) was recognized. Combination chemotherapy including daunorubicin (DNR) and cytosine arabinoside (ara-c) did not subdue the progressive monocytosis and thrombocytopenia. Finally, the patient died of pulmonary hemorrhage 3 months after the onset of the disease.

The prognosis of MDS may be poorly influenced by association with RP.

Association of myelodysplastic syndrome and relapsing polychondritis: further evidence.

Hebbar M, Brouillard M, Wattel E, Decoulx M, Hatron PY, Devulder B, Fenaux P.

Department of Internal Medicine, CHU, Lille, France.

Leukemia 1995 Apr;9(4):731-3 Abstract quote

We report five patients with both a myelodysplastic syndrome (MDS) and relapsing polychondritis (RP), that represented 0.6% of all MDS and 28% of all RP diagnosed over a period of 14 years.

Ten other cases had previously been reported (four in detail), supporting a non-fortuitous association between the two disorders, already suggested for MDS and some other immunological disorders.

Relapsing polychondritis, smouldering non-secretory myeloma and early myelodysplastic syndrome in the same patient: three difficult diagnoses produce a life threatening illness.

Hall R, Hopkinson N, Hamblin T.

Department of Rheumatology and Haematology, Royal Bournemouth Hospital, UK.

Leuk Res 2000 Jan;24(1):91-3 Abstract quote

Multiple myeloma, relapsing polychondritis and myelodysplastic syndrome are all serious diseases in which making a clear diagnosis can be difficult.

This case of a 72-year-old man found after extensive investigation to have all three of the above, demonstrates how difficult diagnosis and treatment can be, producing in this case a life threatening clinical syndrome.

We also postulate that the association of these three diseases may be an immune-derived complication of myelodysplastic syndrome.

CLINICAL APPEARANCE/GROSS DISEASE VARIANTS CHARACTERIZATION

The arthropathy of relapsing polychrondritis.

O'Hanlan M, McAdam LP, Bluestone R, Pearson CM.

Arthritis Rheum 1976 Mar-Apr;19(2):191-4 Abstract quote

Twenty-three adults (11 males and 12 females) with well-defined relapsing polychondritis (RP) are studied in order to characterize the arthropathy of RP. Arthritis was found in 19 patients-as the presenting feature in 8 and as a significant symptom in 11 others. The usual pattern of involvement was migratory, asymmetric, non-nodular, nonerosive, and seronegative, and affected large and small joints as well as parasternal articulations. In addition RP was seen in 3 patients with preexisting chronic polyarthritis or associated rheuamtic disease.

Ocular manifestations of relapsing polychondritis. Three case histories.

Brink H, Rademakers J, Verbeek A, van Ede A, Tilanus S.

Institute of Ophthalmology, Nijmegen University Hospital, The Netherlands.

Doc Ophthalmol 1994;87(2):159-66 Abstract quote

Relapsing polychondritis is a rare, presumably inflammatory autoimmune disorder affecting cartilagenous structures throughout the body. The ears, nose, joints, eyes and the respiratory tract are most frequently involved. The main ocular manifestations are episcleritis and scleritis, conjunctivitis, iridocyclitis and chorioretinitis, cataract and corneal infiltrates and melting. Extraocular signs and symptoms often are indicative of the diagnosis. Therapy includes systemic steroids, immunosuppressive drugs and dapsone.

Three case histories are reported and the literature of ocular manifestations of relapsing polychondritis is reviewed.

Central nervous system involvement in relapsing polychondritis.

Hanslik T, Wechsler B, Piette JC, Vidailhet M, Robin PM, Godeau P.

Service de Medecine Interne, Centre Hospitalier Universitaire Pitie-Salpetriere, Paris, France.

Clin Exp Rheumatol 1994 Sep-Oct;12(5):539-41 Abstract quote

Central nervous system (CNS) involvement is a rare condition in relapsing polychondritis. We report a case of meningoencephalitis that revealed relapsing polychondritis in a 70-year-old woman. A vasculitic process is presumed.

Patient was treated with steroids and dapsone, and no relapse was observed after a follow-up of 2 years.

Stroke as initial manifestation of relapsing polychondritis.

Bouton R, Capon A.

Department of Neurological Rehabilitation, Brugmann Hospital, University of Brussels.

Ital J Neurol Sci 1994 Feb;15(1):61-3 Abstract quote

Relapsing polychondritis (RP) is a rare vasculitide characterized by recurrent inflammation of cartilagenous structures (auricular, nasal, laryngotracheal, articular) and specific sense organs (eyes and ear). Neurological complications are exceptional.

We report the case of an elderly and previously well woman who presented with inflammatory syndrome and stroke as the first manifestations of this disease.

Relapsing polychondritis in childhood--case report and short review.

Knipp S, Bier H, Horneff G, Specker C, Schuster A, Schroten H, Lenard HG, Niehues T.

Department of Paediatrics, Heinrich Heine University of Duesseldorf, Germany.

Rheumatol Int 2000;19(6):231-4 Abstract quote

Relapsing polychondritis (RP) is a disease of unknown etiology and it is characterized by inflammation of the cartilage.

While the clinical picture of RP in adults is well described, RP in childhood is poorly documented. We describe a young girl presenting with acute dyspnea, stridor and polyarthritis. The diagnosis of RP was made 2 years after first presentation, when auricular chondritis occurred. Based on a MEDLINE search, reports on RP in childhood were reviewed. The frequency of chondritis and systemic manifestations of RP in children was compared to data in adults and found to be very similar.

RP in childhood can be a life-threatening and debilitating disease.

HISTOPATHOLOGY CHARACTERIZATION

Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center.

Frances C, el Rassi R, Laporte JL, Rybojad M, Papo T, Piette JC.

Department of Internal Medicine, Hopital de la Pitie, 83, boulevard de l'Hopital, 75651 Paris, France.

Medicine (Baltimore) 2001 May;80(3):173-9 Abstract quote

Dermatologic manifestations of relapsing polychondritis (RP) have been relatively poorly studied compared to other manifestations. In this study we describe dermatologic manifestations in a large series of patients with RP and the corresponding pathologic findings.

In this retrospective, single-center review of 200 patients diagnosed with RP according to Michet's criteria, we analyzed separately those suffering from associated diseases with potential dermatologic involvement or chronic dermatitis. Skin or mucosal biopsies taken from 59 patients were examined without knowledge of the clinical data. Among the 200 patients with RP, 73 had chronic dermatitis or associated diseases with potential dermatologic involvement, especially hematologic disorders (n = 24) and connective tissue diseases (n = 22). Among the other 127 patients, 45 (35.4%) had dermatologic manifestations: aphthosis (n = 21; oral in 14 and complex in 7), nodules on the limbs (n = 19), purpura (n = 13), papules (n = 10), sterile pustules (n = 9), superficial phlebitis (n = 8), livedo reticularis (n = 7), ulcerations on the limbs (n = 6), and distal necrosis (n = 4). Dermatologic manifestations were the presenting feature of RP in 15 cases (12%), and appeared concomitantly (n = 23) or not (n = 22) with attacks of chondritis.

Histologic findings included vasculitis (n = 19, leukocytoclastic in 17 and lymphocytic in 2), neutrophil infiltrates (n = 6), thrombosis of skin vessels (n = 4), septal panniculitis (n = 3), and minor changes (n = 2). Patients with and without dermatologic manifestations did not differ with regard to male/female ratio; age at RP onset; frequency of auricular, nasal, or tracheobronchial chondritis; or frequency of rheumatologic, ocular, audiovestibular, renal, arterial, or venous involvement. The frequency of dermatologic manifestations (91% versus 35.4%; p < 0.0001), sex ratio (18 male/4 female versus 44 male/83 female, p < 0.0001), and age at first chondritis (63.3 +/- 14 yr versus 41.4 +/- 17 yr; p < 0.0002) were significantly higher in the 22 patients with myelodysplastic syndrome than in the 127 patients without any associated disease.

In conclusion, although dermatologic manifestations occur frequently in patients with RP, especially in association with myelodysplasia, they are nonspecific and sometimes resemble those observed in Behcet disease or inflammatory bowel diseases. Their presence in the elderly warrants repeated blood cell counts to detect a smouldering myelodysplasia.

PROGNOSIS AND TREATMENT CHARACTERIZATION

PROGNOSIS

TREATMENT

Relapsing polychondritis: unusual neurological findings and therapeutic efficacy of dapsone.

Ridgway HB, Hansotia PL, Schorr WF.

Arch Dermatol 1979 Jan;115(1):43-5 Abstract quote

Relapsing polychondritis is a rare disease of undetermined cause. The most frequently seen symptom is redness and swelling of the cartilaginous ear, followed by cartilage inflammation elsewhere in the body.

Our five cases had neurologic symptoms that posed diagnostic difficulties. Because vasculitis is not uncommon in relapsing polychondritis, it was difficult to determine whether these neurologic symptoms were attributable to relapsing polychondritis or to other unrelated causes. In our cases, specific neurologic complaints were caused by a concomitant but unrelated disease, rather than by relapsing polychondritis.

We add five additional documented cases to the medical literature and confirm the therapeutic efficacy of dapsone in the treatment of this unusual disease.

Relapsing polychondritis: autoimmunity to type II collagen and treatment with cyclosporin A.

Anstey A, Mayou S, Morgan K, Clague RB, Munro DD.

Department of Dermatology, St Bartholomew's Hospital, London, U.K.

Br J Dermatol 1991 Dec;125(6):588-91 Abstract quote

A case of relapsing polychondritis (RPC) with strongly positive antibodies to both native and denatured type II collagen is reported.

This is only the second report of the use of cyclosporin A in the treatment of RPC.

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Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

Commonly Used Terms

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Internet Links

Last Updated 7/31/2003

Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Prognosis
Treatment
Commonly Used Terms
Internet Links

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSIS
TREATMENT
Relapsing polychondritis: unusual neurological findings and therapeutic efficacy of dapsone. Ridgway HB, Hansotia PL, Schorr WF.	Arch Dermatol 1979 Jan;115(1):43-5 Abstract quote Relapsing polychondritis is a rare disease of undetermined cause. The most frequently seen symptom is redness and swelling of the cartilaginous ear, followed by cartilage inflammation elsewhere in the body. Our five cases had neurologic symptoms that posed diagnostic difficulties. Because vasculitis is not uncommon in relapsing polychondritis, it was difficult to determine whether these neurologic symptoms were attributable to relapsing polychondritis or to other unrelated causes. In our cases, specific neurologic complaints were caused by a concomitant but unrelated disease, rather than by relapsing polychondritis. We add five additional documented cases to the medical literature and confirm the therapeutic efficacy of dapsone in the treatment of this unusual disease.
Relapsing polychondritis: autoimmunity to type II collagen and treatment with cyclosporin A. Anstey A, Mayou S, Morgan K, Clague RB, Munro DD. Department of Dermatology, St Bartholomew's Hospital, London, U.K.	Br J Dermatol 1991 Dec;125(6):588-91 Abstract quote A case of relapsing polychondritis (RPC) with strongly positive antibodies to both native and denatured type II collagen is reported. This is only the second report of the use of cyclosporin A in the treatment of RPC.