No full-text available
To read the full-text of this research,
you can request a copy directly from the author.
Relapsing Polychondritis - Presenting As a Recurrent Auricular Perichondritis-Case Report
... Common infectious etiologies include P. aeruginosa, S. aureus, E. coli, and Proteus species, with P. aeruginosa being the most common [2,3]. If bilateral or recurrent episodes occur, workup for relapsing polychondritis should be entertained [1,4]. ...
CASE PRESENTATION: This is a brief report of a 57-year-old Caucasian female presented with a 4-day history of worsening left ear pain. Her symptoms began with left otalgia and otorrhea which progressed to helical erythema, prompting a visit to the emergency department. She was noted to have erythema of the left auricle and swelling of the left auditory meatus. Our otolaryngology service observed erythema of the auricle with sparing of the lobule.
DIAGNOSIS: The diagnosis to be otitis externa with perichondritis was established, and we recommended otic ciprofloxacin-hydrocortisone, IV vancomycin, and ciprofloxacin. The patient had marked improvement and was discharged on an oral and otic fluoroquinolone. In this case, the diagnosis of perichondritis was made by a classic physical examination finding: erythema and edema with sparing of the fatty lobule. This key finding helps to distinguish perichondritis from otitis externa.
The intravenous injection of crystalline papain into young rabbits results in depletion of cartilage matrix throughout the body, with loss of rigidity and collapse of the ears, provided the enzyme is inactivated by oxidation or sulfhydryl blocking agents prior to administration. Cysteine-activated crystalline papain, when injected intravenously, produces little or no change in cartilage.
The changes which occur in cartilage following an injection of inactivated crystalline papain are indistinguishable from those produced by crude papain.
Activation of crude papain by cysteine prior to injection results in loss of its capacity to produce in vivo changes in cartilage.
The progressive changes which take place in cartilage in vivo also occur in vitro in isolated rabbit ears removed shortly after an injection of crude papain or inactivated crystalline papain. In vitro ear collapse occurs rapidly at 37°C. and does not occur at 4°C. Collapse is enhanced by exposing the cartilage to cysteine and prevented by exposure to iodoacetamide or p-chloromercuribenzoate.
The direct action of crystalline papain on plates of normal cartilage, in vitro, results in the same gross and histological changes which were observed in vivo. The direct action is accelerated by cysteine and inhibited by iodoacetamide or p-chloromercuribenzoate.
The intravenous injection of iodoacetamide-treated bromelin produces the same in vivo changes in cartilage as papain. Untreated bromelin has no demonstrable effect on cartilage.
It is suggested that the reason for the failure of activated papain to enter cartilage, after being injected intravenously, is that it probably reacts with a substrate or substrates in the blood. Oxidized or otherwise inactivated papain, in contrast, is readily taken up by cartilage and there converted to its active form.
A 57-year-old man and a 70-year-old woman with relapsing polychondritis are reported. The man, suffering from arthralgias, respiratory obstruction, external ear and sanddle-nose deformities, conjunctivitis and irido-cyclitis, died after 4 years from airway obstruction because of tracheal and bronchial collapse. The woman is alive 8 months after the development of respiratory obstruction, probably caused by radiographically demonstrated tracheal obstruction, a saddle-nose deformity and hearing impairment. Microscopically, the involved cartilages showed degenerative and slight inflammatory changes and were eventually replaced by fibrous tissue. Histochemical studies, utilizing staining with Alcian blue at controlled electrolyte concentrations (Scott technique) and at controlled pH:s, with or without digestion with bacterial chondroitinase ABC; and staining with the PAS-method, with or without diastase digestion, revealed a complete or relative loss of glucosaminoglycans and glycogen. A biosynthetic defect is considered unlikely to be the primary pathogenetic mechanism of relapsing polychondritis. Histological and histochemical examination of biopsies from involved cartilages contribute to a definite diagnosis.
Relapsing polychondritis is a rare disease of unknown etiology. There are approximately 211 reported cases in the world literature. This is a report of ten cases from the Cleveland Clinic Foundation.
McAdam's diagnostic criteria for R.P. were reviewed and modified. For diagnosis, all patients had to have 1. at least three or more diagnostic criteria, histologic confirmation not necessary; 2. one or more of McAdam's signs with positive histologic confirmation; or 3. chondritis in two or more separate anatomic locations with response to steroids and/or Dapsone.
Chondritis of the auricles (9/10 patients) and arthropathy (8/10 patients) are the most common presenting signs. Chondritis was also seen in the nose (6/10) and the upper respiratory tract involving the larynx and trachea (4/10). Cochlear and vestibular damage and ocular inflammation were each seen in 5/10 patients.
Patients were treated with steroids and/or Dapsone. Both drugs were reliable in abating episodes of activity and in decreasing recurrences. These results further support Dapsone as an alternate form of treatment for RP.
Relapsing polychondritis (RP) is not a totally rare rheumatic disease. We have seen 23 patients from 1960-1975, and there are now a total of 159 reported cases, which form the basis of this study. RP occurs equally in both sexes, and has a maximum frequency in the fourth decade. 2) Empirically defined diagnostic criteria are proposed, to include the most common clinical features: a) Bilateral auricular chondritis b) Nonerosive sero-negative inflammatory polyarthritis c) nasal chondritis d) Ocular inflammation e) Respiratory tract chondritis f) Audiovestibular damage The diagnosis is based primarly upon the unique clinical features, and is quite certain if three or more criteria are present together with histologic confirmation. 3) Fifty percent of patients present with either auricular chondritis or the arthropathy of RP; but with prolonged follow-up, a majority of patients develop four or more of the above mentioned criteria. 4) Approximately 30 percent of patients have a preceding or coexistent rheumatic or autoimmune disease, which can lead to initial diagnostic confusion. 5) Laboratory and radiographic investigations help mainly to rule out other diagnostic possibilities, with no characteristic abnormalities being present in a majority of patients. 6) On follow-up, three-fourths of our patients required chronic corticosteroid therapy with an average dose of 25 mg per day of prednisone. Corticosteroids decrease the frequency, duration, and severity of flares, but do not stop disease progression in severe cases. 7) The mortality rate has been 30 percent in our series and 22 percent in the other 136 reported cases. Of the 29 cases where the cause of death was known, 17 were from respiratory tract involvement and 9 from cardiac valvular or vasculitic involvement, emphasizing the need to search for critical involvement of either of these organ systems in each patient. 8) Detailed reports of selected cases are presented to illustrate the clinical diagnosis and differential diagnosis, and to demonstrate the need for careful prolonged follow-up. 9) Although the etiology remains unknown, there is a frequent association with, and clinical similarity to, other rheumatic diseases. 10) Careful clinicopathological study of our 23 patients leads us to postulate an underying systemic vascultis as an important pathologic mechanism in RP.
Relapsing polychondritis is a systemic disease of unknown etiology with predominate manifestations of multiorgan cartilaginous inflammation. Although relapsing polychondritis occurs predominantly as a separately defined clinical complex, a significant number of patients may suffer from another rheumatic disease. Vasculitic syndromes are the most commonly observed disorders associated with relapsing polychondritis.
We present the case history of a 50-year-old man with seropositive erosive rheumatoid arthritis of 30-years standing who developed polychondritis simultaneously with several extra-articular rheumatoid manifestations, such as anaemia, subcutaneous nodules, pericarditis and episcleritis. The relevant literature is reviewed. Gradually, all symptoms and signs disappeared after start of treatment with 30 mg prednisone and 100 mg azathioprine daily. We suggest that the polychondritis in this patient was also an extra-articular manifestation of rheumatoid arthritis.
Three cases of relapsing polychondritis are described. One patient had polyarthritis, scleritis, and chondritis of both pinnae. Another had chondritis of his nose and pinnae, serous otitis media, episcleritis and aortitis causing aortic regurgitation: he survived aortic valve replacement but eventually died from a ruptured aneurysm of the abdominal aorta. The third patient had chondritis of the right pinna and nose, polyarthralgia and iritis in association with an illness resembling a systemic vasculitis and including severe proliferative glomerulonephritis. New chemical and histo-chemical data demonstrating a uniform loss of all classes of glycosaminoglycans (mucopolysaccharides) from the affected cartilage and aorta are presented as evidence against a primary biochemical defect. A review of these and the 111 previously reported cases show relapsing polychondritis to have the character of an acquired disease related to the collagen disorders and vasculitides. Anti-cartilage antibodies were demonstrated in the serum of two of the three patients by the indirect immunofluorescent technique.
Relapsing polychondritis (RP) is an autoimmune disorder with chondritis as a prominent clinical feature. This disease has been found to coexist in some patients with systemic lupus erythematosus (SLE) or vice versa. We report a patient with clinical criteria fulfilling the diagnosis of RP. She had elevated levels of anti-dsDNA and decreased levels of CH50 during the 6-month observation, without clinical manifestations of SLE except for arthritis. We also reviewed all 16 cases of previously described patients with RP and SLE. From our study, we conclude that chondritis is rare in patients with SLE. If immunologic abnormalities usually found in SLE are detected in patients with RP, clinicians should observe patients for other manifestations of SLE.
Relapsing polychondritis: Immunomicroscopic findings in cartilage of ear biopsy specimens
- R Valunzuela
- P A Cooperrider
- P Gogate
Valunzuela R, Cooperrider PA, Gogate P (1980) Relapsing polychondritis: Immunomicroscopic findings in cartilage of ear biopsy specimens.
Human Pathol 11: 19.