No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Policondritis recidivante: a propósito de un caso
Abstract
Presentamos un caso de policondritis recidivante que debutó con hipoacusia y cursó con ciertas peculiaridades. La paciente fue diagnosticada en un principio de otitis serosa. Investigando más detenidamente el caso se encontraron hallazgos que condujeron a la sospecha de policondritis recidivante. Se confirmó el diagnóstico mediante biopsia del cartílago nasal, realizándose a continuación un estudio sistémico exhaustivo. Como características peculiares del caso encontramos una hipoacusia mixta cuyo componente neurosensorial revirtió parcialmente con corticoides.
... Além do pavilhão auricular, o ouvido externo, médio e interno podem ser atingidos. O ouvido externo é envolvido pela extensão da inflamação ao canal auditivo externo; o médio, pela obstrução tubárica; e o interno, por fenómenos de vasculite dos vasos cocleo-vestibulares 10 . A condrite nasal ocorre em 54% dos casos, podendo conduzir ao colapso da cartilagem, com deformação «em sela» do nariz 8 . ...
The authors describe the case of a 49 year-old male patient with a 3-year history of antiphospholipid syndrome, admitted after presenting in the emergency room with erythematous nodular skin lesions, affecting the face and neck, with a week's duration. Local biopsies were suggestive of interstitial granulomatous dermatitis. The patient described lesions compatible with bilateral auricular chondritis, two weeks prior to the appearance of the nodules, which resolved spontaneously after 3 days. There was a previous episode of nasal chondritis, two years previously, and another episode starting at the 7th day of hospitalization. These findings, taken together with a diagnosis of seronegative polyarthritis established 5 years before the current events, lead to a diagnosis of relapsing polychondritis.
Relapsing polychondritis (RP) is a rare autoimmune disease that affects cartilage and multiple organ system. We describe a case of RP presenting with fever, conjunctivitis and inflammatory signs of the right knee. Infectious and neoplastic diseases were excluded by imaging and laboratory examinations. After 17 days of admission the patient reported coughing and odynophagia, and, a physical examination showed red ears. Otorhinolaryngological examination demonstrated arytenoid chondritis. RP was diagnosed based on four McAdam's criteria. The patient was medicated with oral prednisone, and a positive clinical response was noted. Severe disease may require high doses of corticosteroids or immunosuppressive agents and the biological treatment may be beneficial. Beginning manifestations, such as fever, are often unspecific, leading to RP misdiagnosis. In this case, close attention to the patient's clinical history and a detailed physical examination were fundamental in concluding the correct diagnosis, and consequently beginning the appropriate medication.
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.
Collagen molecules are major extracellular matrix proteins involved in the development and support of delicate auditory sensory organs. Type II collagen is widely distributed within inner ear tissues, while type IX is found only within the labyrinthine membrane and dense fibers of the tectorial membrane. Antibody specific for type II collagen has been shown to be elevated in some patients with hearing loss due to several presumably autoimmune illnesses (including Meniere's disease, otosclerosis, chronic progressive sensorineural hearing loss, and relapsing polychondritis). Purified human type II and IX collagens and an extract of human cochlear tissue were subjected to isolation by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and transferred to nitrocellulose. The sera of 21 patients with inner ear disease were examined for the presence of anticollagen and anticochlear antibodies; the sera were used to probe Western blots of purified human collagens II, IX, and XI, and cochlear protein extract with peroxidase-conjugated goat anti human polyvalent immunoglobulin as the second antibody. Anti-type II collagen antibodies were seen in 12 of 21 (57%) patients, while 13 of 21 (62%) had anti-type IX antibodies detectable by Western blot. A previously unreported 30 kd (probably noncollagen) protein was 21 (62%) had anti-type IX antibodies detectable by Western blot. A previously unreported 30 kd (probably noncollagen) protein was found by SDS-PAGE of human cochlear tissue extracts, with 3 patients, all with Meniere's disease, having antibody activity to this protein detected by Western blot.(ABSTRACT TRUNCATED AT 250 WORDS)
Relapsing polychondritis is not a very frequent systemic disease. Nevertheless, it has to be borne in mind since very often the first symptoms appear in the sphere of otorhinolaryngology. We are faced with the problem of differential diagnosis in a case which in the beginning seemed to be a neurological pattern. The definitive diagnosis was not established until 15 months after the appearance of the symptoms when, after several outbreaks, the clinical pattern was clearer. As we have to deal with a recurrent and systemic pathology whose diagnosis is eminently clinical, long-term pursuit and valuation are necessary to allow us to know the natural history of the disease as well as the reaction to the treatment.
Inflammatory destruction of cartilages, presumably on an autoimmune basis, characterizes relapsing polychondritis, a disease of varying severity and outcome. The otolaryngologist-head and neck surgeon is often the first physician contact, because the preponderance of signs and symptoms are in the head and neck, ie, auricular and nasal chondritis, ocular and auditory manifestations, and laryngotracheobronchial involvement.
A sixty-five year old woman developed relapsing polychondritis with three of the diagnostic criteria established by McAdam et al. (1976), namely bilateral auricular chondritis, ocular inflammation and both cochlear and vestibular dysfunction. Many authors have mentioned other neurological symptoms including unilateral facial weakness. This patient is the first case described with bilateral facial weakness and cerebral manifestations.
Relapsing polychondritis, an uncommon, recurrent, inflammatory disorder affecting the cartilaginous tissues of the body (Jaksch-Wartenhorst, 1923; Herman, 1981), is regularly associated with audiovestibular dysfunction (Bollet et al., 1969; Cody et al., 1971; McAdam et al., 1976; Ridgway et al., 1979). McAdam et al. (1976) and Ridgway et al. (1979) have mentioned other neurological symptoms including unilateral facial weakness. We recently observed a patient with polychondritis and multifocal neurological abnormalities. We believe this is the first case described with bilateral facial nerve palsy and with cerebral manifestations.
Relapsing polychondritis is a rare inflammatory disease that causes destruction of cartilaginous tissue in various anatomical regions. We report here about a 55-year-old female patient with relapsing polychondritis that involved the right auricle, both audiovestibular organs and both eyes. The patient presented with persisting inflammation of the right auricle, sudden lower-frequency hearing loss, acute moderate vertigo with nausea and mild ocular symptoms. Immunofluorescence assays were used for the detection of antibodies against the cochlea and the vestibular organ and demonstrated the presence of circulating antibodies against the audiovestibular organ. No staining for anti-corneal IgG was detected. Improvement of clinical disease was achieved by treatment with systemic steroids and vasodilator drugs, and long-term medication with low-dose corticosteroids.