1/16. 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 = rare disease (Clic here for more details about this article) |
2/16. Laryngotracheal involvement of relapsing polychondritis in a Korean girl.We describe a young girl presenting with acute dyspnea because of laryngotracheal involvement of relapsing polychondritis (RP). RP, a multisystemic disorder of unknown etiology, is a very rare disease in children. It is characterized by inflammation and destruction of the cartilaginous structure of many organs, including the respiratory tract. Early respiratory tract involvement in younger patients is the greatest threat to life, and aggressive therapy, including tracheostomy and intravenous high-dose steroids, is advocated.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/16. Relapsing polychondritis--two cases with tracheal stenosis and inner ear involvement.Relapsing polychondritis is a rare disease which often presents firstly to ear, nose and throat (ENT) departments. Its complications, respiratory, cardiovascular, renal and neurological are life-threatening; thus it is important to recognize the disease and its complications early. Treatment for relapsing polychondritis may have serious side-effects which should be taken into account when managing these patients. We report two young patients with relapsing polychondritis and their treatments; both had severe tracheal stenosis responding in one case to pharmacological and in the other to surgical intervention.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/16. Relapsing polychondritis: reversible airway obstruction is not always asthma.Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/16. Relapsing polychondritis: a rare disease with varying symptoms.PURPOSE: Relapsing polychondritis (RPC) is a rare systemic disease affecting primarily cartilaginous and proteoglycan-rich structures. It is a potentially fatal disease with unknown aetiology. There are no specific tests for RPC. The diagnosis is dependent on clinical criteria, which include chondritis of both auricles, non-erosive inflammatory polyarthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis and cochlear and/or vestibular damage. Ocular symptoms will occur in approximately 60% of RPC patients. As an example, a patient with signs of RPC is described. methods/RESULT: A 30-year-old woman was referred to our department for evaluation of a central corneal ulcer in the left eye. She had a history of recurrent pain in both her auricles and was also found to have a nasal septum perforation. Relapsing polychondritis was suspected. CONCLUSION: Non-healing corneal ulcers should alert the ophthalmologist to look for unusual reasons for this condition. RPC is one possible cause.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
6/16. A case of relapsing polychondritis preceded by inner ear involvement.Relapsing polychondritis is a rare disease of unknown etiology causing systematic inflammation of the whole cartilaginous tissues. We report a patient with relapsing polychondritis preceded by inner ear involvement. A 68-year-old female visited our hospital for a chief complaint of sudden bilateral hearing loss. On pure-tone audiometry, sensorineural hearing loss, 47dB on the right and 51dB on the left, was observed. Later, bilateral auricular chondritis and uveitis developed. On biopsy of the auricular cartilage, perichondritis was observed. The patient was diagnosed with relapsing polychondritis in the early stage, 2 weeks after the onset. Oral predonine administration was initiated, and the auricular inflammation was improved within 10 days. The hearing loss disorder was gradually improved. The steroid dose was reduced, and no decrease in hearing has been observed so far.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/16. Aseptic meningitis and ischemic stroke in relapsing polychondritis.Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous structures. Its involvement of central nervous system is uncommon. We report the case of a 71-year-old woman whose initial manifestations were RP and meningitis and whose subsequent attack of RP was associated with a left cerebral infarction. The patient responded to steroid therapy in both attacks of RP.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/16. 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 = rare disease (Clic here for more details about this article) |
9/16. Relapsing polychondritis with severe aortic insufficiency.Relapsing polychondritis (RP) is a rare disease characterized by auricular, nasal and respiratory tract chondritis, ocular inflammation, inflammatory polyarthritis and cardiovascular abnormalities. Here we describe a patient with a five-year history of mild nasal and auricular chondritis, which suddenly developed into a severe aortic insufficiency with rest dyspnea. The pathogenesis and the management of this rare connective tissue disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/16. Narrowing of the airway in relapsing polychondritis.Relapsing polychondritis, once thought to be a very rare disease, is being recognized with increasing frequency. It is characterized by inflammation of cartilaginous structures throughout the body. In some cases, the eye and ear are involved; aortic aneurysms develop in a minority of patients. The cartilages of the airway are affected in more than 50% of all cases, and the resulting stenotic lesions can be life-threatening. Radiographic study of the airway is of great value in detection and evaluation of upper respiratory involvement.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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