1/235. A man with inflamed ears. A 23-year-old man presented with fever, dyspnea, nonproductive cough, left eye redness, reduced vision, and bilateral ear pain and tenderness. The symptoms had begun two days earlier, eight days after he was discharged from the hospital with a presumptive diagnosis of Still's disease. He was first seen a month before the current admission for complaints of fever (as high as 39.4 degrees C), nonproductive cough, and asymmetric arthritis. The workup at that time included arthrocentesis of the right knee. Analysis of the joint fluid showed 7,500 white blood cells/mm3 and no crystals. A gram stain and culture of the fluid were negative. hiv and hepatitis tests, bone marrow biopsy and culture, transesophageal echocardiography, abdominal computed tomography, radionuclide bone scanning, and rheumatologic tests failed to identify the problem. The development of an evanescent macular pink rash on day 15 suggested the possibility of Still's disease. Treatment with prednisone (40 mg po qd) was initiated, and the patient was discharged on day 19. ( info) |
2/235. 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. ( info) |
3/235. 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. ( info) |
| 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. ( info) |
5/235. 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. ( info) |
6/235. Endobronchial stenting for severe airway obstruction in relapsing polychondritis. Airway complications of relapsing polychondritis (RP), including tracheobronchial stenosis, can be fatal. This paper describes a life-saving technique (placement of multiple metallic endobronchial stents under conscious sedation) to prevent life-threatening airway closure in a 50-year-old woman with RP. Using fluoroscopic and bronchoscopic guidance, a tracheal stent and three endobronchial metallic stents were deployed in the central airways, with good functional outcome. There were no complications. In critical airway compromise caused by RP, the insertion of endobronchial stents can result in improved symptoms, pulmonary function, and a return to daily activities, without the use of tracheotomy and mechanical ventilation. ( info) |
7/235. Management of airway manifestations of relapsing polychondritis: case reports and review of literature. STUDY OBJECTIVE: To report the first series of patients with severe airway manifestations of relapsing polychondritis (RP) that were managed successfully with self-expandable metallic stents, and to review the literature. DESIGN: Retrospective review of medical records, and current clinical follow-up. SETTING: Tertiary care referral hospital. patients: All patients with airway manifestations of RP that were managed with self-expandable metallic stents at our institution. RESULTS: All five patients (four women and one man; age, 40 to 69 years old) had severe airway manifestations, and three of them required mechanical ventilation. spirometry with flow-volume curves showed severe combined obstructive and restrictive ventilatory defects. bronchoscopy revealed dynamic collapse of the proximal airways. diagnosis was made 8 months to 13 years after the first symptom of the disease. Pharmacotherapy included prednisone, methotrexate, cyclosporine, and dapsone. A total of 17 self-expandable metallic stents of varying sizes were placed using flexible bronchoscope from 4 to 19 years after the first symptom. The overall outcome was favorable in four patients. Three patients have survived without ventilatory support 16 to 18 months following the first stent placement, and the fourth patient survived for 20 months without ventilatory support before she died. The fifth patient, who was receiving mechanical ventilation, died in 1 week probably due to persistent dynamic collapse of the airways distal to the stents. CONCLUSION: Self-expandable metallic tracheobronchial stents should be considered in the management of airway manifestations of RP, especially in patients who require mechanical ventilation. ( info) |
8/235. 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. ( info) |
9/235. 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. ( info) |
10/235. Relapsing polychondritis and myelodysplasia: a report of two cases and review of the current literature. Relapsing polychondritis (RP) is a rare multisystem disorder. We describe two case reports of patients with RP, one of whom developed myelodysplasia subtype refractory anaemia (RA) and the other, refractory anaemia with ringed sideroblasts (RARS). We also review the literature of association between RP and haematological disorders. ( info) |