Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Relapsing Polychondritis Introduction Relapsing polychondritis (RP) is an episodic systemic disorder characterized by recurrent, widespread, and potentially destructive inflammatory lesions involving cartilaginous structures, the cardiovascular system, and organs of special sense such as the eyes and ears. Attacks tend to vary in severity and duration usually lasting for days to weeks before resolving spontaneously. Protracted episodes may lead to permanent loss of cartilaginous structural integrity. The first known description of RP comes from Dr. Jaskch-Wartenhorst who in 1923 wrote in the Vienna Archives of Internal Medicine about his experiences with a young brewery worker who presented to him with fever and painful arthritic swellings of his hand, knee and foot. Several months later the man developed painful swellings of both auricles that subsided spontaneously leaving deformities and shortly thereafter developed a spontaneous saddle-nose deformity. Dr. Jaskch-Wartenhorst called this disorder "polychondropathia" speculated that the disorder may have had some relation to the excessively large quantities of beer that his patient consumed. Today this disorder is classified among the autoimmune connective tissue disorders because of its clinical and mechanistic similarity to other autoimmune diseases. It is unique and of special interest to the Otolaryngologist because of the preponderance of head and neck manifestations of the disease and because we are often the first to see and have a chance to diagnose this disorder. The average age at onset for this disorder is 41 - 51 years; there is no sex or race predilection and it can occur at any age. The histopathology reveals cartilage destruction with loss of basophilic staining and islands of lymphocytic infiltration; later there is fragmentation of cartilage with replacement by fibrous tissue. It is believed that autoantibodies to cartilage components, specifically type II collagen cause inflammatory infiltration and cellular mechanisms involving lysosomal enzyme release eventually result in destruction of the cartilage. Diagnostic Criteria Three or more clinical signs must be present:
OR Chondritis in 2 or more separate sites AND response to steroids or immunosuppression Differential diagnosis for these individual symptoms and signs includes most of the autoimmune disorders and other infectious and non-infectious granulomatous disorders including Wegener's granulomatosis, PAN, Takayasu's, GCA, rheumatoid arthritis, Reiter's, rheumatic fever, polymorphic reticulosis, syphilis, TB, histoplasmosis, leprosy, sarcoidosis, and malignancy. Clinical Symptoms and Signs Presenting symptoms are most frequently auricular chondritis, polyarthritis, nasal chondritis, ocular inflammation, or respiratory tract involvement. Overall the following findings occur: auricular chondritis (89%), polyarthritis (81%), nasal chondritis (72%), ocular inflammation (65%), respiratory tract involvement (56%), audio-vestibular symptoms (46%), cardiac and vascular manifestations (24%) and skin lesions (17%). The auricle in RP is classically painful, erythematous and edematous with characteristic sparing of lobule. Cauliflower deformity may result with calcification of cartilaginous portions. Middle ear may be involved with eustachian tube dysfunction and collapse resulting in serous otitis media or conductive hearing loss. Respiratory system involvement is among the more serious manifestations of RP. Edema, stenosis, chondomalacia, collapse and disintegration of the airway cartilages results in laryngo-tracheo-bronchial chondritis, chondromalacia, subglottic stenosis causing collapse of trachea and bronchi, pulmonary infections and, finally, asphyxia. CT scan may be useful in assessing respiratory involvement of disease. The auditory-vestibular System may become involved (less common) with SNHL; believed to be a cochlear loss due to arteritis of the internal auditory artery. Steroid boost may help recover hearing. Vertigo usually appears in conjunction with SNHL and is also due to arteritis of internal auditory artery. Cardiovascular manifestations, systemic and CNS arteritis (11%), aortic aneurysm (5%), valvular insufficiency (7%), and pericarditis occur in 24%; the histopathology is similar to cystic medial necrosis with elastic layer destruction. A sero-negative, non-erosive polyarthropathy may be observed; involvement of all 3 cartilage types distinguishes this from rheumatoid arthritis. Costochondral, peripheral or spinal joints may be involved. Patients may also experience costochondral junction pain (arthropathy). The nasal cartilage may become involved with acute chondritis (pain, swelling), rhinorrhea, epistaxis, or saddle nose deformity. Ocular findings include episcleritis (most common), conjunctivitis, iritis and scleritis. Sixteen percent of patients eventually develop cutaneous lesions: cutaneous vasculitis, erythema nodosum and a variety of nonspecific lesions. Renal disease (glomerulonephritis) is distinctly uncommon and suggests concurrent systemic vasculitis or other associated disease. Approximately 25% of cases have coexistent diseases, especially autoimmune diseases (systemic vasculitis (PAN, Takayasu's, GCA, Wegener's), rheumatoid arthritis, Sjogren's syndrome, SLE, PSS, Reiter's, Behcet's, thyroid disease, ulcerative colitic and paraneoplastic syndrome). No specific laboratory test for RP exists and findings are often non-specific. The following serologic results may be found: elevated ESR (86% of patients), leukocytosis (38%), anemia (57%), elevated ASO titer (29%), RF (17%), ANA (18%), eosinophillia (14%), serogic test for syphilis (6%) and LE prep (8%). Radiographic studies are mostly useful in delineating complications by showing destruction of cartilage. Treatment and Prognosis Treatment consists of symptomatic care for mild cases with anti-inflammatory agents. Dapsone has also been used with success. More severe cases, especially those with potentially life-threatening complications require steroids or even immunosuppressive agents. Azothiaprime, cyclophosphamide, methotrexate, and cyclosporin A have all been used in efforts to control the course of this disease. Natural history is unpredictable and may have an episodic, smoldering or fulmanent course. Mortality is usually secondary to: respiratory complications (airway collapse or pneumonia); cardiovascular complications (aneurysm rupture, vasculitis or valvular heart disease); infectious complications related to immunosuppression due to treatment; or concurrent malignancy. Survival probability is 74% overall, but 45% for those with vasculitis (similar to vasculitis alone). RP directly accounts for 48% - 86% of deaths. Anemia is a poor prognostic indicator for all ages. Poor prognostic indicators for patients less than 50 years or age include saddle-nose deformity, arthritis, laryngotracheal strictures, vasculitis, and microhematuria. For patients older than 50 years old, the number of disease manifestations correlated with survival. Case Presentation A previously healthy 35-year-old Hispanic man who presented to the Emergency Center with a 4-month history of intermittent, crampy, epigastric pain and bilaterally swollen, painful ears. He had three previous EC visits for these problems and had been treated for presumed gastritis and bilateral external otitis with Pepcid and Cortisporin Otic drops, but had not experienced relief of his symptoms. He was admitted to the hospital for further evaluation. On admission he incidentally reported a 30 pound weight loss, nightly chills, dizziness, hearing loss, and painful conjunctivitis. On examination he was also noted to have fever of 101.6oF, bilaterally edematous and erythematous pininas, and costochondral tenderness. Laboratory testing was significant for microcytic anemia and elevated liver function. His HIV and a hepatitis screen were negative. A full abdominal work-up was negative for significant pathology. An ophthalmologic examination revealing episcleritis and anterior chamber uveitis prompted further diagnostic testing. An ESR was significantly elevated at 98 and RF, ANA, ANCA, RPR and MHA-TP were all negative. Based on clinical criteria, he was diagnosed with polychondritis and started on high dose steroids. He was discharged from the hospital after showing improvement in his symptoms. One month later he presented to the Rheumatology service with stridor and shortness of breath. An otolaryngology consultation was obtained and he was found to have a severe subglottic stenosis clearly visible on flexible laryngoscopic examination. CT scanning of his neck revealed chondromalacia and subglottic stenosis. After informed discussion with the patient, a tracheostomy was performed. Direct laryngoscopy and bronchoscopy at that time confirmed the clinical and radiographic findings. Biopsies were consistent with the diagnosis of polychondritis. Bibliography Anstey A, Mayou S, Morgan K, Clague RB, Munro DD. Relapsing polychondritis: autoimmunity to type II collagen and treatment with cyclosporin A. Br J Dermatol 1991;125:588-591. Batsakis JG, Manning JT. Relapsing polychondritis. Ann Otol Rhinol Laryngol 1989;98:83-84. Booth A, Dieppe PA, Goddard PL, Watt I. The radiological manifestations of relapsing polychondritis. Clin Radiol 1989;40:147-149. Campbell SM, Montanaro A, Bardana EJ. Head and neck manifestations of autoimmune disease. Am J Otolaryngol 1983;4:187-216. Carrion M, Giron JA, Ventura J, Camacho A, Garcia-Diez C. Airway complications in relapsing polychondritis. J Rheumatol 1993;20:1628-1629. 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Van Besien K, Tricot G, Hoffman R. Relapsing polychondritis: a paraneoplastic syndrome associated with myelodysplastic syndromes. Am J Hematol 1992;40:47-50. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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