Cases reported "Polychondritis, Relapsing"

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1/8. Orbital mucosa-associated lymphoid tissue (MALT)-type lymphoma in a patient with relapsing polychondritis.

    Relapsing polychondritis is characterized by recurrent inflammation of the cartilaginous tissues of the ears, nose, peripheral joints, and the tracheobronchial tree. The eye is also a frequent target organ in relapsing polychondritis, and proptosis is a well-recognized manifestation of eye involvement. Similar to other rheumatologic diseases, an association of relapsing polychondritis with malignancy has been reported. We describe a patient with relapsing polychondritis who presented with exophthalmos. When treatment directed toward control of her underlying disease was only partially effective, further investigation revealed that she had an orbital mucosa-associated lymphoid tissue (MALT)-type B cell lymphoma. We hypothesize that the lymphoma resulted from malignant transformation of the relapsing polychondritis-induced inflammatory pseudotumor and emphasize that neoplastic disease should be considered in the differential diagnosis in patients with relapsing polychondritis presenting with exophthalmos.
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2/8. Relapsing polychondritis studied by computed tomography.

    Computed tomographic findings in a patient with relapsing polychondritis are described. Collapse of the cartilage of the nose and calcification in cartilages of the ears were clearly demonstrated. CT scanning was also helpful in evaluating the tracheobronchial tree for airway compromise, which could prove fatal in this condition.
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3/8. Use of metallic stents in relapsing polychondritis.

    Relapsing polychondritis is a rare multisystem disease. We describe the presentation and treatment of a patient with relapsing polychondritis and review the literature. This patient had involvement of the tracheobronchial tree requiring insertion of metallic stents.
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4/8. Computed tomography of relapsing polychondritis.

    The CT findings of a patient with relapsing polychondritis involving the larynx, tracheobronchial tree, and nasal cartilage are described. In the proper clinical setting, a characteristic constellation of findings when noted on CT can aid in differentiating this rare inflammatory disease from other causes of airway compromise. CT is the most useful imaging modality, because cartilage and soft tissue components are well visualized. If diagnosed early, appropriate treatment may prevent life-threatening airway obstruction.
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5/8. Severe endobronchial obstruction in a girl with relapsing polychondritis: treatment with Nd YAG laser and endobronchial silicon stent.

    Relapsing polychondritis (RP) is an uncommon disorder of unknown aetiology characterized by inflammation and destruction of the cartilaginous structures of many organs, including the tracheobronchial tree. When untreated, there is a high mortality rate, usually from respiratory obstructive complications. An 8 year old white girl, with a previous diagnosis of RP, was referred to our department for evaluation of worsening dyspnoea. bronchoscopy showed localized inflammatory and fibrotic alterations of the mucosa, leading to severe obstruction of the left mainstem bronchus at its origin. The condition was successfully treated by endoscopic neodymium yttrium aluminium garnet (Nd YAG) laser. Re-evaluation of the patient, 7 months later, demonstrated bronchial stenosis and malacia requiring mechanical dilatation and positioning of an endobronchial silicon stent, which was well-tolerated by the patient.
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6/8. Three-dimensional imaging of the pediatric airway.

    Accurate imaging of the pediatric tracheobronchial tree is indicated for the evaluation of congenital or acquired abnormalities. Conventional axial computed tomography (CT) is now considered the best imaging modality for evaluation of the trachea and major bronchi, and has almost completely replaced the former gold standard of tracheobronchography. Preliminary results indicate that CT scan performance is further enhanced through the application of spiral technology and two-dimensional (2D) and 3D representation of the tracheobronchial tree. Spiral CT scans with 3D surface rendering offers an opportunity to replace traditional tracheobronchography with a safer, less invasive modality.
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7/8. A case of relapsing polychondritis presenting as mediastinal syndrome, diagnosed by CT scans of the trachea and head.

    OBJECTIVE: The aim of this study is to evaluate the significance of CT scans of the trachea and head in the diagnosis of Relapsing Polychondritis (RP). DESIGN: Relapsing polychondritis is a disease involving cartilaginous structures, particularly those of the ears, nose and trachea. diagnosis is based on specific clinical features and immuno-histopathological evaluation of the cartilages involved. SETTING AND patients: We describe a case of RP in which the most evident clinical signs (cough, dyspnoea, vertigo, tinnitus, headache, oedema of the face and shoulders and fever), led us first to suspect a mediastinal compression syndrome. INTERVENTION: A CT scan of the trachea and head revealed details which established the correct diagnosis, supported by other typical RP symptoms and by histopathological examination of the cartilage. MAIN OUTCOME MEASURES: Evaluation by CT scan of the chest, the mediastinum, the head and the pinnae. RESULTS: CT scanning revealed thickening and calcification of the anterolateral tracheal wall and main bronchi besides marked narrowing of the trachea. CT of the head showed calcification also of the external auditory meatus and part of the pinnae. CONCLUSION: We consider that CT scan of the trachea and head is helpful in evaluating the bronchial tree, the auditory meatus and pinnae as well as being a valid tool for the final diagnosis and in following the course of the disease.
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8/8. Relapsing polychondritis.

    In this article, we report on a 40-year-old man with relapsing polychondritis (RP) of the tracheobronchial tree without clinical manifestations of other systems including nasal septum and earlobe cartilage involvement. The illness was diagnosed histologically, and treatment with procaine penicillin was successful. RP is an unusual systemic disorder characterized by widespread inflammation and destruction of cartilage tissues. The main cause is usually autoimmune. In RP various clinical manifestations including nasal chondritis, arthritis, scleritis, damage to tracheal and bronchial cartilage, and aortic, cardiac, and renal involvement may occur. Isolated tracheobronchial involvement is very rare. The diagnosis must be based on a combination of clinical and pathologic features. If diagnosed early, appropriate treatment may prevent life-threatening airway obstruction.
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