1/55. Anti-labyrinthine antibodies in a patient with relapsing polychondritis.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.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
2/55. Relapsing polychondritis diagnosed by Tc-99m MDP bone scintigraphy.PURPOSE: Relapsing polychondritis is a generalized recurring disease of cartilage that involves joints, trachea, bronchi, laryngeal cartilages, costal cartilages, and cartilages of the ear and nose. It is associated with autoimmune diseases, including hashimoto disease in some cases. methods: The authors evaluated a 29-year-old man with relapsing polychondritis who had symptoms and signs of a common cold for 2 months and anterior chest pain near the sternum for 1 month. RESULTS: After the diagnosis, the authors found that the patient had a history of thyroid therapy for hyperthyroidism 15 years before. Tc-99m MDP bone scintigraphy performed to evaluate anterior chest pain showed diffusely increased accumulation of radioactivity in all costocartilages and sternoclavicular joints. Based on that information, relapsing polychondritis was diagnosed. Ga-67 citrate scintigraphy was preformed to determine the optimum biopsy site of the cartilage. The diagnosis was histologically supported by the results of the open biopsy. CONCLUSIONS: In this case, Tc-99m MDP bone scintigraphy was useful for diagnosing relapsing polychondritis, and Ga-67 citrate scintigraphy was helpful in determining the biopsy site.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
3/55. A new role for magnetic resonance imaging in the diagnosis of laryngeal relapsing polychondritis.BACKGROUND: Relapsing polychondritis involving the upper airway is a rare cause of airflow obstruction and hoarseness. The diagnosis of relapsing polychondritis depends on clinical signs, characteristic findings on cartilage biopsy, and response to treatment. Delays in diagnosis and treatment can increase the morbidity and mortality of the disease. methods: We present a case report of primary laryngeal relapsing polychondritis. RESULTS: Serologic testing, direct laryngoscopy, and endoscopic biopsy could not establish the diagnosis of relapsing polychondritis. magnetic resonance imaging (MRI) examination demonstrated findings consistent with the histopathological diagnosis obtained on open biopsy. Follow-up MRI after treatment showed resolution of the initial findings. CONCLUSION: This case demonstrates the usefulness of MRI in the diagnosis and management of relapsing polychondritis involving the upper airway.- - - - - - - - - - ranking = 20.279178430619keywords = mortality, rate (Clic here for more details about this article) |
4/55. A case of relapsing polychondritis involving the tragal and the conchal bowl areas with sparing of the helix and the antihelix.We describe a 65-year-old white man with a 21-year history of recurrent, afebrile episodes of painful, tragal, conchal bowl and eyelid swelling accompanied by occasional conjunctivitis. The remainder of the auricle was not involved. Episodes were both self-remitting and responsive to intramuscular steroid injections. Cutaneous and cartilaginous tissues were examined histologically following a therapeutic debulking procedure. The histologic features included dermal edema, vascular dilatation, and small vessel inflammation with a dense polymorphous inflammatory infiltrate rich in eosinophils. Perichondrial inflammation and cartilage degeneration with fibrosis were characteristically observed. Bacterial cultures demonstrated normal flora. This case fulfills the revised diagnostic criteria of relapsing polychondritis. It demonstrates an unusual presentation within the disease spectrum of relapsing polychondritis with tragal and conchal bowl involvement and sparing of the helix and the antihelix.- - - - - - - - - - ranking = 1.5keywords = rate (Clic here for more details about this article) |
5/55. When is a biopsy justified in a case of relapsing polychondritis?Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.- - - - - - - - - - ranking = 0.22869123592502keywords = death (Clic here for more details about this article) |
6/55. Relapsing polychondritis, smouldering non-secretory myeloma and early myelodysplastic syndrome in the same patient: three difficult diagnoses produce a life threatening illness.multiple myeloma, relapsing polychondritis and myelodysplastic syndrome are all serious diseases in which making a clear diagnosis can be difficult. This case of a 72-year-old man found after extensive investigation to have all three of the above, demonstrates how difficult diagnosis and treatment can be, producing in this case a life threatening clinical syndrome. We also postulate that the association of these three diseases may be an immune-derived complication of myelodysplastic syndrome.- - - - - - - - - - ranking = 0.5keywords = rate (Clic here for more details about this article) |
7/55. Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis.PURPOSE: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/methods: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. RESULTS: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure. CONCLUSIONS: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.- - - - - - - - - - ranking = 0.95738247185004keywords = rate, death (Clic here for more details about this article) |
8/55. Exudative retinal detachment in relapsing polychondritis : case report and literature review.OBJECTIVE: To report the atypical ocular symptoms (arterialized conjunctival vessels, exudative retinal detachment) that can be the presenting manifestations of relapsing polychondritis. DESIGN: Observational case report and literature review. methods: A complete ocular and systemic evaluation was performed on a patient with relapsing polychondritis and exudative retinal detachment. MAIN OUTCOME MEASURES: Retinal, choroidal, and scleral findings. RESULTS: A 73-year-old man with relapsing polychondritis presented with a unilateral large bullous exudative retinal detachment and marked choroidal and scleral thickening bilaterally. CONCLUSIONS: Ophthalmologists should consider relapsing polychondritis in the differential diagnosis of exudative retinal detachment. A combination of echographic and laboratory findings can assist in the accurate diagnosis of this rare condition.- - - - - - - - - - ranking = 0.5keywords = rate (Clic here for more details about this article) |
9/55. Otolaryngological aspects of relapsing polychondritis: course and outcome.Relapsing Polychondritis (RP) is a rare disorder characterised by episodic inflammation of the cartilaginous structures. Differential diagnosis of this pathology is a challenging enigma since it appears only intermittently and none of the clinical features are pathognomonic, although most of the patients are usually referred to an ENT physician initially. The natural history of the disease is unpredictable. Initiating effective treatment sooner considerably reduces the rate of mortality. patients with RP should be seen on a regular basis even if the patient is non-symptomatic. This study reports a retrospective analysis of the otolaryngological manifestations of 7 patients with RP as well as their long-term progress and reviews the symptomatology, histopathology, immunology and management.- - - - - - - - - - ranking = 19.779178430619keywords = mortality, rate (Clic here for more details about this article) |
10/55. Identification of type II collagen peptide 261-273-specific T cell clones in a patient with relapsing polychondritis.OBJECTIVE: To characterize and clone T cells specific for type II collagen (CII) in a patient with relapsing polychondritis (RP) and to establish whether the immunodominant epitope of CII determined in HLA transgenic mice is used in the human autoimmune response to CII. methods: T cell responses to CII were examined in a patient with RP, who was heterozygous for the HLA-DR allele DRB1*0101/DRB1*0401. T cell clones were established from this patient and characterized for peptide specificity, class II restriction, cytokine production, and staining with HLA-DRB1*0401 class II tetramers. RESULTS: A response to CII and the peptide 255-273 was present in this patient. T cells specific for the CII epitope 261-273 were cloned. Evaluation of these clones demonstrated a response to CII 261-273 in the context of both DR alleles. HLA-DR4 CII tetramer did not demonstrate staining of either CII-specific DRB1*0401-restricted T cell clones or a polyclonal population of CII-reactive T cells from this individual. CONCLUSION: T cells directed against CII were present in this patient with RP. Also, T cell clones isolated from this individual were found to be specific for the CII peptide 261-273 and were restricted to either the DRB1*0101 or the DRB1*0401 allele. These findings establish that a T cell response directed against CII is present in this patient with RP and that the CII peptide 261-273 plays a role in the human immune response to CII.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
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