1/16. Relapsing polychondritis.BACKGROUND: Relapsing polychondritis (RPC) is a rare, chronic, and potentially fatal multisystemic inflammatory disorder targeting cartilaginous structures. This disorder is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and/or hematologic disorders. RPC afflicts patients with recurrent and often progressive episodes of inflammation with the potential for destruction of the affected structures. tissues involved include the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and skin. Ocular manifestations commonly include episcleritis, scleritis, conjunctivitis, iridocyclitis, chorioretinitis, and proptosis. Lid edema, orbital inflammation, muscle palsies, and corneal melting may also occur. CASE REPORT: An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. After orbital cellulitis was ruled out, further evaluation revealed posterior scleritis with choroidal detachment OS. A course of oral indomethacin and topical antibiotic-steroid combination drops was implemented in the treatment of the ocular manifestations. The quick positive response to the anti-inflammatory agents confirmed the diagnosis of ocular complications secondary to RPC. DISCUSSION: The presenting ocular signs and symptoms of RPC often resemble other commonly encountered ocular conditions. It is important for the eye care practitioner to be familiar with the ocular manifestations of RPC because the eyes are sometimes the initial site of involvement and may be a marker of severity. early diagnosis and intervention may significantly improve the patient's outcome. This case report with literature review will hopefully bring to light features of this disease which will help the eye care practitioner in the diagnosis and management of this condition.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
2/16. Active aortitis in relapsing polychondritis.Relapsing polychondritis (RP) is a rare inflammatory multiorgan disorder affecting cartilaginous structures and other connective tissues. Serious cardiovascular complications have been reported in patients with RP, the most frequent being aortic or mitral regurgitation and aortic aneurysms. aortitis is a very rare complication. An unusual case of active aortitis in a patient with RP, despite intensive immunosuppressive treatment, is described with a special emphasis on the pathological findings.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
3/16. pulmonary fibrosis with intractable pneumothorax: new pulmonary manifestation of relapsing polychondritis.Relapsing Polychondritis is a rare disease which causes the repetitive inflammation of cartilage and connective tissues. Although the large airway is sometimes involved and the stenosis of them often influences the prognosis of the patients, there have been few reports concerning the manifestation of the peripheral lung. A 60-year-old man with pulmonary fibrosis was admitted to a regional hospital due to sudden deafness, and then he suffered from relapsing polychondritis. During the steroid therapy, he also suffered from bilateral pneumothoraces. His computed tomogram revealed many bilateral bullae, emphysematous changes, and fibrotic changes in bilateral lungs. The mechanism of generating peripheral pulmonary manifestations is also discussed.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
4/16. Anesthetic management of a patient with relapsing polychondritis--a case report.Relapsing polychondritis is a rare multisystem disorder of uncertain etiology. It is characterized by recurrent and progressive destruction of both elastic and hyaline cartilages as well as connective tissue. Respiratory tract involvement is associated with high mortality and morbidity. General anesthesia may be required in these patients for tracheostomy, bronchoscopy, nasal reconstruction, aortic valve replacement, and recent tracheobronchial stenting which they usually sustain. tracheostomy was once the most likely surgical procedure in relapsing polychondritis. However, this procedure is only effective in patients with upper subglottic involvement. In cases of extensive tracheobronchial involvement, tracheostomy is ineffective because the distant tracheal collapse below the tracheostomy is still unresolved. We would like to report a case of relapsing polychondritis with tracheobronchial involvement, who underwent an emergent tracheostomy. tracheostomy with continuous positive airway pressure (CPAP) effectively improved her airway collapse. Herein, we also discuss the anesthetic management after review of the current literature.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
5/16. Relapsing polychondritis, chronic hepatitis c virus infection, and mixed cryoglobulemia.OBJECTIVE: review of relapsing polychondritis (RP) and its association to chronic hepatitis c virus (HCV) infection and mixed cryoglobulinemia. methods: A case of RP associated with HCV infection is reported. The English language medical and scientific literature was reviewed for RP, hepatitis c, and its relation to other connective tissue diseases from February 1966 to January 2003 using medline. RESULTS: RP is an uncommon, multisystem disease of unknown etiology characterized by recurrent inflammation of cartilaginous and related tissues, being associated with other diseases in 30% to 35% of cases. HCV infection is a systemic illness with a propensity to trigger or exacerbate autoimmune disorders: eg, essential mixed cryoglobulinemia, membranoproliferative glomerulonephritis, and leukocytoclastic and systemic vasculitis. We could find no previous report of an association between RP with HCV and mixed cryoglobulinemia. Treatment with interferon gamma and ribavirin (IR) not only induced an undetectable viral load, but also resolved symptoms of RP. CONCLUSIONS: We report a patient with RP, HCV, and mixed cryoglobulinemia. It is unknown if there is a cause-effect or chance relationship. Treatment with IR improved the symptoms of RP. It is not known whether the effects of IR were directly on the RP or suppressed RP indirectly through the actions on the viral load or active hepatitis.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
6/16. Relapsing polychondritis in hiv-infected patients: a report of two cases.Relapsing polychondritis is a rare autoimmune disease characterized by inflammation and degeneration of cartilaginous tissue. We describe the first reported cases of relapsing polychondritis in patients with human immunodeficiency virus (hiv) and no associated connective tissue diseases. The relationship between autoimmune and hiv diseases is complex and unclear. Treatment of hiv disease with antiretroviral therapy and subsequent immune restoration may lead to the development of autoimmune diseases in genetically susceptible individuals.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
7/16. Meningeal plasma cell granuloma with relapsing polychondritis. Case report.Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51-year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
8/16. Relapsing polychondritis and Reiter's syndrome.We describe a case of relapsing polychondritis associated with Reiter's syndrome in a 46-year-old white man. Relapsing polychondritis is a rare disorder of unknown etiology associated in about 30% of cases with other rheumatic disorders such as connective tissue diseases, vasculitis and exceptionally, seronegative spondyloarthritis. We found this association only once in our review of the literature.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
9/16. Relapsing polychondritis--a report on two Chinese patients with severe costal chondritis.Relapsing polychondritis is a rare multisystemic connective tissue disorder found mainly in Caucasians. Oriental patients with relapsing polychondritis are uncommon. We report 2 Chinese patients with relapsing polychondritis who had severe laryngotracheal narrowing requiring tracheostomy. Unlike most patients, there was marked costal chondritis resulting in depression of the anterior chest wall with minimal involvement of other joints. One of the patients had treatment with prednisolone, dapsone, azathioprine, cyclophosphamide and cyclosporin A at various stages of the disease without significant remission of the disease.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
10/16. Thoracic epidural morphine in the palliation of chest wall pain secondary to relapsing polychondritis.Relapsing polychondritis is a rare disease of unknown etiology characterized pathologically by degeneration of the chondrocyte and replacement with fibrous connective tissue. The following case report presents the pain management of a 34-yr-old man suffering from intractable pain secondary to relapsing polychondritis. Systemic narcotic analgesics, adjunctive drugs, and peripheral nerve blocks with local anesthetic and steroid failed to adequately control the patient's pain. Thoracic epidural morphine was used to provide excellent relief of pain. Factors in the selection of an implantable narcotic delivery system as well as practical considerations including tolerance and potential side effects of intraspinal narcotics are discussed. Ethical issues surrounding the chronic use of intraspinal narcotics in the setting of chronic benign pain are also discussed.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
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