Polychondritis
Kitty Kelly
November 17, 1994
Carla M. Giannoni, M.D.
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:
Recurrent chondritis both auricles
Non-erosive inflammatory polyarthritis
Nasal chondritis
Ocular inflammation
Respiratory tract chondritis
Cochlear and, or, vestibular dysfunction
OR 1 or more signs with histologic confirmation
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.
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.
Clark LJ, Wakeel RA, Ormerod Ad. Relapsing polychondritis - two cases
with tracheal stenosis and inner ear involvement. J Laryngol Otol
1992;106:841-846.
Cody DT, Sones DA. Relapsing polychondritis: audiovestibular
manifestations. Laryngoscope 1971;81:1208-1222.
Coppola M, Yealy DM. Relapsing polychondritis: an unusual cause of
painful auricular swelling. Ann Emerg Med 1992;21:81-85.
Damiani JM, Levine HL. Relapsing polychondritis - report of ten cases.
Laryngoscope 1979;89:929-946.
Eng J, Sabanathan S. Airway complications in relapsing polychondritis.
Ann Thorac Surg 1991;51:686-692.
Foidart IM, Abe S, Martin GR, Zizic TM, Barnett EV, Lawley TJ, et al.
Antibodies to type II collagen in relapsing polychondritis. N Engl J Med
1987;299:1203-1207.
Gaffney RJ, Harrison M, Blayney AW. Nebulized racemic ephedrine in the
treatment of acute exacerbations of laryngeal relapsing polychondritis.
J Laryngol Otol 1992;106:63-64.
Handrock K, Gross WL. Relapsing polychondritis as a secondary phenomenon
of primary systemic vasculitis. Ann Rheum Dis 1993;52:895-897.
Herman JH. Polychondritis. Curr Opin Rheumatol 1991;3:28-31.
Herman J. Polychondritis. In: Kelley WN, Harris ED Jr, Ruddy S, Sledge
CB, eds. Textbook of Rheumatology. Philadelphia: Saunders, 1981.
Hussain SS. Relapsing polychondritis presenting with stridor from
bilateral vocal cord palsy. J Laryngol Otol 1991;105:961-963.
Irani BS, Martin-Hirsch DP, Clark D, Hand DW, Vize CE, Black J.
Relapsing polychondritis - a study of four cases. J Laryngol Otol
1992;106:911-914.
Joliat T, Seyer J, Bernstein J, Krug M, Ye XJ, Cho JS, et al. Antibodies
against a 30 kilodalton cochlear protein and type II and IX collagens in
the serum of patients with inner ear diseases. Ann Otol Rhinol Laryngol
1992;101:1000-1006.
Kurien M, Seshadri MS, Raman R, Sen-Bhanu T. Inherited nasal and
laryngeal degenerative chondropathy. Arch Otolaryngol Head Neck Surg
1989;115:746-748.
McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing
polychondritis: prospective study of 23 patients and a review of the
literature. Medicine 1976;55:193-215.
Michet CJ Jr, McKenna CH, Luthra HS, O'Fallon WM. Relapsing
polychondritis: survival and predictive role of early disease
manifestations. Ann Inter Med 1986;104:74-78.
Michet CJ. Vasculitis and relapsing polychondritis. Rheum Dis Clin North
Am 1990;16:441-444.
Papo T, Piette JC, Le-Thi-Huong-Du, Godeau P, Meyer O, Kahn MF, et al.
Antineutrophil cytoplasmic antibodies in polychondritis. Ann Rheum Dis
1993;52:384-385.
Pearson CM, Kline HM, Newcomer VD. Relapsing polychondritis. N Engl J
Med 1960;263:51-58.
Port JL, Khan A, Barbu RR. Computed tomography of relapsing
polychondritis. Comput Med Imaging Graph 1993;17:119-123.
Specks U, Wheatley CL, McDonald TJ, Rohrbach MS, DeRemee RA.
Anticytoplasmic autoantibodies in the diagnosis and follow-up of
Wegener's granulomatosis. Mayo Clin Proc 1989;64:28-36.
Van Besien K, Tricot G, Hoffman R. Relapsing polychondritis: a
paraneoplastic syndrome associated with myelodysplastic syndromes. Am J
Hematol 1992;40:47-50.
® Copyright, 1996. All Rights Reserved.
Baylor College of Medicine. Bobby R. Alford Department of
Otorhinolaryngology and Communicative Sciences.
Harvey R. Stone
wkmo...@gmail.com
lindalt39
> thanks Kitty.
>
> Kitty Kelly wrote:
> >
> > RELAPSING POLYCHONDRITIS
>
> >
Linda Tribout