Relapsing polychondritis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ,Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Historical Perspective

• In 1923, Jaksch-Wartenhorst used the term polychondropathia to describe his first case about relapsing polychondritis.
• In 1960, the term relapsing polychondritis was first coined by Pearson.

Classification

• There is no established classification of relapsing polychondritis.

Pathophysiology

• The pathogenesis of relapsing polychondritis is unknown.
• Several studies have suggested the role of both cellular immunity and abnormal autoantibody in the pathogenesis of relapsing polychondritis.
• The HLA class II has been associated with the development of relapsing polychondritis, there is increase susceptibility with HLA-DR4.
• On gross pathology, thickening of cartilage of ear, nose, epiglottis, and cricoid and tracheal rings are characteristic findings of relapsing polychondritis.
• On microscopic histopathological analysis of relapsing polychondritis, following features are seen:
  • Lymphocytes, plasma cells, neutrophils, and eosinophils are seen in the cartilage.
  • Cartilage has lost the chondrocytes and normal architecture at the later stages of inflammation.
  • Cartilage is replaced by fibrous tissue in the end.

Differentiating Relapsing Polychondritis from other Diseases

• Chondritis
• Chondromdermatitis
• Hansen's disease
• Nodularis helicis
• Perichondritis
• Rhinophyma
• Skin cancer
• Syphilis

Epidemiology and Demographics

• The incidence of relapsing polychondritis is approximately 4 per 100,000 individuals in Minnesota.

Age

• Relapsing polychondritis is more commonly observed among patients aged 40 to 60 years but it can also occur in childhood.

Gender

• Relapsing polychondritis affects men and women equally.

Race

• Relapsing polychondritis usually affects Caucasian.

Risk Factors

Common risk factors in the development of relapsing polychondritis include:

• Genetic susceptibility:
  • Increased risk with HLA DR4
• History of other connective tissue disorders

Natural History, Complications and Prognosis

• The majority of patients with relapsing polychondritis remain asymptomatic for several years.
• Early clinical features include ear pain, polyarthritis and other non-specific symptoms such as fever, weight loss and skin rash.
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

Mc Adam diagnostic criteria for relapsing polychondritis
Criteria	Requirement
Recurrent chondritis of both auricles	3 out of 6 features are required for definitive diagnosis of relapsing polychondritis
Non-erosive inflammatory polyarthritis
Nasal chondritis
Inflammation of auricular structures
Chondritis of the respiratory tract
Cochlear and/or vestibular damage

According to modified Damiani criteria, there should be one of the following findings to diagnose relapsing polychondritis:

• There should be three of McAdam's diagnostic criteria.
• One or more of the clinical findings of McAdam's diagnostic criteria and positive histologic confirmation.
• Chondritis at two or more separate anatomic locations with a response to glucocorticoids or dapsone.

Symptoms

Symptoms of relapsing polychondritis may include the following:

• Ear pain
• Eye pain, redness, and increased lacrimation
• Decreased visual acuity
• Proptosis
• Rhinorrhea
• Epistaxis
• Pain on the movement of joint
• Joint swelling
• Cough
• Shortness of breath
• Dysphagia
• Chest pain
• Headache
• Confusion

Physical Examination

• Patients with relapsing polychondritis usually appear lethargic.
• Physical examination may be remarkable for:
• Ear examination:
  • On inspection, there is inflammation and swelling
  • On palpation, there is tenderness
• Eye examination:
  • On inspection, lid retraction, redness of the eye, ptosis, and proptosis
  • Visual acuity is decreased
• Lung examination:
  • On auscultation, wheezing and expiratory stridor is found
• Heart examination:
  • S1, S2 is normal, the murmur can be heard depending on the involvement of valve
• Joint examination:
  • On inspection, there is redness and swelling
  • On palpation, there is tenderness

Laboratory Findings

• There are no specific laboratory findings associated with relapsing polychondritis.
• Abnormal laboratory findings consistent with the diagnosis of relapsing polychondritis include:
  • CBC with differentials
    • Low hemoglobin
    • Leukocytosis
    • Eosinophilia
  • ESR and CRP are usually raised
  • Antibody testing
    • Anti-type II collagen antibodies is usually raised in early phase of disease
    • Antineutrophil cytoplasmic antibodies is found in patients who has vasculitis
  • Urinary glycosaminoglycans levels may be elevated
  • Urinary collagen type II neoepitope levels is elevated in active inflammation
    • Levels of urinary collagen type II neoepitope is used to assess response to treatment
  • Serum levels of cartilage oligomeric matrix protein is elevated and used as a marker of disease activity

Imaging Findings

• X-ray of the chest shows tracheal stenosis.
• CT scan of the chest shows following findings:
  • Subglottic stenosis
  • Thickening of anterior and lateral tracheal wall with sparing of the posterior membranous wall

Relapsing polychondritis
Source: Case courtesy of Dr Charlie Chia-Tsong Hsu

• MRI is better than CT scanning to see the difference between edema, fibrosis, and inflammation

Treatment

Medical Therapy

• The mainstay of therapy for relapsing polychondritis is medical therapy.
• NSAIDs are used as an initial treatment of relapsing polychondritis.
• Preferred regimen: Naproxen 500 mg PO q12h or ibuprofen 800 mg PO q6h x 7 to 10 days.
• If NSAIDs are contraindicated or resistant then following regimens are used:
  • Preferred regimen: Dapsone 50 to 100 mg PO q24h x 4 months depending on the severity of the disease.
  • Preferred regimen: Prednisone 30 to 60 mg PO q24h in divided dose.

Treatment of relapse:

• Preferred regimen: Prednisone 5 to 7.5 mg PO q24h

Treatment of life-threatening organ damage:

• Preferred regimen: Initial therapy is the combination of prednisone 1 mg/kg PO q24h and cyclophosphamide 2 mg/kg PO q24h.
• Maintenance therapy depends on the renal function of a patient.
• Patient without nephritis, Preferred regimen: Methotrexate 15mg PO once weekly.
• Patient with nephritis, Preferred regimen: Azathioprine

Surgery

Medical therapy is the mainstay of therapy for relapsing polychondritis, however various surgical options include:

• Tracheostomy
• Cardiac valve replacement
• Aortic aneurysm repair
• Saddle-nose deformity repair

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

External links

• Polychondritis Educational Society, Ltd. (PES)
• Dr. D. E. Trentham research paper
• MedicineNet.com
• The Polychondritis Group - Support Group

Template:Osteochondropathy Template:Diseases of the musculoskeletal system and connective tissue