Recurrent polychondritis M94.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Askanazy Syndrome; Chondromalacia systematized; Generalized chondrolytic perichondritis; Panchondritis; Perichondritis generalized chondrolytic; Polychondritis chronica atrophicans; Polychondritis recidivans et atrophicans; recurrent polychondritis; Relapsing Polychondritis; Rheumatism of the cartilage system; Systematized chondromalacia; Von Meyenburg-Altherr-Uehlinger syndrome; Von-Meyenburg-Altherr-Uehlinger syndrome

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Jaksch-Wartenhorst, 1923; Altherr and Meyenburg, 1936

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Rare, inflammatory rheumatic systemic disease with preferential attack of the articular and non-articular cartilage with chondrolysis, dystrophy and atrophy. Leading symptoms are auricular chondritis and nasal chondritis as well as arthralgias and arthritides (in 1/3 of patients as initial symptoms). Furthermore, there is a possible infestation of the hyaline articular cartilage as well as the fibrous cartilage of intervertebral discs.

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Very rare, incidence is 0.35/100.00/year; in < 30% association with other systemic diseases (rheumatic diseases, vasculitis, collagenosis, Sjögren's syndrome); sex distribution: f:m = 7:10 (n=158 cases). The disease occurs in all ethnic groups with a low incidence in Caucasians.

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Unknown, probably autoimmune disease with autosensitization to cartilage tissue (collagen type 2?). Associations with HLA DR 4.

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Mainly occurring between the 4th and 6th decade of life (average age: 45.3 years). Possible at any age.

Clinical features
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Frequently begins with inflammation of the ear cartilage (> 80%): Recurrent inflammation of the auricles lasting for days or weeks; these are bright red and painful. Later consequences are "cauliflower or washcloth ears". Characteristic is the "remaining free" of the earlobes (no cartilage!).

Catarrh of the upper airways, flu-like infections, fatigue, shortness of breath (softening of the cartilage in the larynx and trachea (31-67%).

Frequent nasal involvement: inflammation of the nasal cartilage is often associated with epistaxis and rhinorrhoea. Later development of a "saddle nose".

Polyarthritis (rheumatoid factor negative!) in 80% of cases with characteristic rheumatoid complaints. The arthritis is asymmetrical, relapsing, wandering and does not show a uniform correlation to the extra-articular complaints.

Inflammatory eye infections: conjunctivitis, episcleritis, iridocyklitis, retinitis (49-65% of cases).

Vascular involvement: aortic aneurysm and insufficiency.

Neurological symptoms (2-8% of cases)

Kidney affections (7-26% of cases)

Skin infestation: vasculitis (4-38%)

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SPA elevation, leukocytosis, reactive protein positive. Urine: acid mucopolysaccharides elevated, proteinuria.

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Loss of basophilia of cartilage, inflammatory lymphocytic infiltrates, cartilage fragmentation, fibrosis. The epidermis is unremarkable.

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Diagnostic criteria (quoted from Krumbholz et al.):

  1. Recurrent chondritis of the ear cartilage (cauliflower ear)
  2. Non-erosive seronegative polyarthritis
  3. Chondritis of the nasal cartilage (saddle nose)
  4. Eye infestation (50% of cases: episcleritis, iridocyclitis)
  5. chondritis of the respiratory tract
  6. Infestation of the audiovestibular system.
  • Not listed are fever and general symptoms

According to McAdam: For the diagnosis 3 of the 6 criteria must be fulfilled. According to Damiani: 3 of the 6 criteria must be met. Alternative: 1 criterion must be fulfilled + histological evidence of chondritis or 2 criteria must be fulfilled + response to glucocorticoids or dapsone.

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  • Cartilage destruction of e.g. the bronchial system.
  • Combinations with other diseases (approx. 1/3 of cases): systemic vasculitis, chronic polyarthritis, juvenile rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, Reiter's disease, psoriatic arthritis, progressive systemic scleroderma, thyroid diseases (Hashimoto's thyroiditis), ulcerative colitis, primary biliary cirrhosis.

Internal therapy
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  • Glucocorticoids: First choice. Initial glucocorticoids like prednisone (e.g. Decortin) 40-60 mg/day p.o., reduction according to clinic to 5-25 mg/day p.o. Cave! Stomach protection, e.g. with Riopan Gel.
The following drugs have been described as alternatives or supplements with varying degrees of success:

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Chronic course over 3-5 years. The prognosis is serious, it depends on the involvement of the respiratory tract (infections) and the heart. 5-year survival rate 75%

Case report(s)
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Case 1. The 76-year-old single pensioner noticed within 4 days a bright red, pressure-painful swelling of the right auricle. No fever, no chills, no swelling of the lymph nodes. Pre-existing conditions: 3rd degree AV block, laryngeal swelling with vocal cord paralysis two years ago, erythematous swelling of the nose 1 year ago.
  • Laboratory: CRP 38.2 mg/l (slightly elevated), leucocytes 10,200/μl; antistreptolysin titre 80.4 IU/ml (normal range), rheumatoid factor neg.; no detection of collagen II antibodies.
  • Course: At first the diagnosis is mainly erysipelas. Cave! Typical misdiagnosis.
  • Therapy: Trial with 5 million IU penicillin G sine effectu 3 times/day, including increasing swelling of the ear. In case of critical evaluation of the symptoms revision of the suspected diagnosis: recurrent polychondritis.
  • Histology (HE staining): In the upper dermis low perivascular lymphocytic infiltrates, dense towards the depth. Cartilage inconspicuous. DIF: Interstitial deposits of IgG, fibrinogen,C3.
  • Therapy: Prednisolone 100 mg/day p.o. (gradual reduction to 25 mg within 14 days), Azathioprine 2 times/day 50 mg p.o. Within 2 days sudden (!) clinical improvement. Pat. was discontinued to long-term therapy (prednisolone 5 mg/day and azathioprine 100 mg/day) with satisfactory results.
Case 2: 53-year-old female patient complained for about 3 months of recurrent painful swelling and redness of the left auricle, occasionally also of the right auricle. Furthermore, there was occasional swelling and pain in the right ankle and wrist, passagere hearing disorders as well as phased redness of the left eye.
  • Dermatological findings: On contact, a flat, highly painful, deep red swelling of the left auricle, with the earlobe remaining sharply defined excluded.
  • Other clinical findings: Reddening of the left eye (ophthalmological findings: iridocyclitis). The right wrist was swollen and painful with limited movement (skeletal scintigram: significant increase in the number of patients).
  • Histology The epidermis is unremarkable. Shaken lymphocytic perivascularly oriented infiltrates; incised cartilage: loss of basophilia of cartilage, inflammatory lymphocytic infiltrates, cartilage fragmentation.
  • Laboratory: BSG-70/120 mm nw; CRP: 150mg/l; 12.00 leucocytes/ml; proteinuria. Antibodies against collagen II negative.
  • X-Ray-Thorax findings o. B.
  • Cardiac echo No evidence of cardiac involvement
  • Diagnosis: Based on the typical clinical picture with complaints of different organ systems (here: ear with typical recess of the earlobe, eye, joints) and considering the history and histology, the diagnosis "recurrent polychondritis" was made.
  • Therapy and course: Prednisolone therapy (starting with 150mg/day and degressive dosage, consecutive therapy 15mg/day with overlapping long-term therapy of azathioprine 1,5mg/kgkgKG); including prompt and clear improvement of all symptoms. During the 6-month follow-up there is a stable finding with at most minor deformity of the ear.

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    China and review of the literature. Rheumatol Int 36:1003-1009.
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  10. Vroman D et al (2003) Images in clinical medicine. Relapsing polychondritis. N Engl J Med 349: e3


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Last updated on: 29.10.2020