• Archives of Craniofacial Surgery
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• v.20 no.5
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• pp.341-344
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• 2019

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.

• Tuberculosis and Respiratory Diseases
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• v.80 no.4
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• pp.351-357
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• 2017

In 2011, a cluster of peripartum patients were admitted to the intensive care unit of a tertiary hospital in Seoul with signs and symptoms of severe respiratory distress of unknown etiology. Subsequent epidemiological and animal studies suggested that humidifier disinfectant (HD) might represent the source of this pathology. Epidemiological studies, animal studies, and dose-response analysis demonstrated a strong association between HD use and lung injuries. The diagnostic criteria for HD-associated lung injury (HDALI) was defined on the basis of the clinical, pathological, and radiological attributes of the patients. The clinical spectrum of HDALI appears to range from asymptomatic to full-blown acute respiratory failure, and some patients have required actual lung transplantation for survival. The overall mortality of the exposed population was not significant, although peripartum patients and children who were admitted to the intensive care unit did show high mortality rates. Persistent clinical findings such as diffuse ill-defined centrilobular nodules and restrictive lung dysfunction were observed in some of the survivors. The findings of this review emphasize the importance of assessment of the level of toxicity of chemical inhalants utilized in a home setting, as well as the need to identify and monitor afflicted individuals after inhalational injury.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.798-804
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• 1996

Chronic eosinophilic pneumonia is a very rare disorder of unknown etiology characterized by striking systemic and pulmonary manifestations such as fever, weight loss, dyspnea, blood eosinophilia, and fluffy peripheral opacities on chest radiograph. A number of these patients developed asthma before or with the onset of illness. The roentgenographic lesion rapidly resoluted with corticosteroid and recurrence was occasionally occured in the same location. Histopathologic features of chronic eosinophilic pneumonia include dense aggregates of eosinophils, histiocytes, and multinucleated giant cells within alveolar spaces, interstitium, and bronchioles associated with scattered lymphocytes and plasma cells. We report a case of chronic eosinophilic pneumonia diagnosed by clinical, radiographic, and histologic findings with review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.71 no.1
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• pp.55-58
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• 2011

Pulmonary alveolar microlithiasis is a rare disease of unknown etiology that is characterized by the presence of calcific concentrations in the alveolar spaces. The radiographic appearance is pathognomonic. Plain chest radiographs show a white lung or sandstorm lung consisting of fine sand like microcalcifications diffusely scattered throughout both lungs with a higher density at the lung bases. We now report the case of a 67-year-old male whose diagnosis was based on characteristic findings on a chest X-ray and a high-resolution computed tomography scan.

• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.373-378
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• 2001

An idiopathic mediastinal fibrosis is a rare disease with an unknown etiology. It is a benign condition in which a fibrosis of the soft tissue and chronic inflammation occurs within the mediastinum. This leads to a constriction and obliteration of the adjacent mediastinal structures, particularly the great veins. This can result in a variety of clinical conditions depending on the anatomic location of the disease. Here, we report a case of an idiopathic mediastinal fibrosis with vocal cord palsy, which was confirmed by a biopsy with a thoracotomy. Postoperative medical treatment using prednisolone and tranilast was performed.

• Tuberculosis and Respiratory Diseases
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• v.40 no.4
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• pp.431-435
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• 1993

Relapsing polychondritis is a rare and sometimes fatal disease of unknown etiology, manifestating as an episodic and progressive inflammatory disorder, affecting predominantly the cartilage of the ears, nose and tracheobroncheal tree as well as internal structures of the eyes and ears. We experienced a case of relapsing polychondritis involving the larynx, tracheobroncheal tree, eye, nose and ear in a 20 year old male, required tracheostomy due to severe dyspnea.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.856-860
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• 1999

Diffuse pulmonary ossification is rare disease of unknown etiology. Since the first description by Luschka in 1856, about 140 cases have been reported worldwide, but no such case has been reported in Korea yet. We report 40-year-old woman who was diagnosed as diffuse nodular pulmonary ossification on open lung biopsy. She has no respiratory symptoms & physical findings and no previous disease history. She was incidentally found to have multiple pulmonary nodules on roentgenographic examination. Open lung biopsy was done for above lesion and She was proven to have diffuse nodular pulmonary ossification. She was followed by roentgenographic examination and showed no progression.

• Tuberculosis and Respiratory Diseases
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• v.42 no.2
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• pp.231-237
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• 1995

Chronic sclerosing mediastinitis is a rare disease of unknown etiology, pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. The process is often progressive and can occur either focally or diffusely throughout the mediastinum. This can result in compression of adjacent mediastinal structures, most commonly the low-pressure superior vena cava but also the pulmonary artery and vein, trachca and bronchi, esophagus and can result in a variety of functional and roentgenographic manifestation and occasionally death. We experienced a case of chronic sclerosing mediastinitis of unknown cause, which was confirmed by biopsy with thoracotomy, so reported it with a review of literature.

• Tuberculosis and Respiratory Diseases
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• v.75 no.3
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• pp.116-119
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• 2013

Sarcoidosis, a systemic granulomatous disease of unknown etiology. The presentation of sarcoidal granuloma in neck nodes without typical manifestations of systemic sarcoidosis is difficult to diagnose. We describe the case of a 37-year-old woman with an increasing mass on the right side of neck. The excisional biopsy from the neck mass showed noncaseating epithelioid cell granuloma of the lymph nodes. No evidence of mycobacterial or fungal infection was noted. Thoracic evaluations did not show enlargement of mediastinal lymph nodes or parenchymal abnormalities. Immunohistochemistry showed abundant expression of tumor necrosis factor-${\alpha}$ in the granuloma. However, transforming growth factor-${\beta}$ was not expressed, although interleukin-$1{\beta}$ was focally expressed. These immunohistochemical findings supported characterization of the granuloma and the diagnosis of sarcoidosis. Sarcoidosis can present with cervical lymph node enlargement without mediastinal or lung abnormality. Immunohistochemistry may support the diagnosis of sarcoidosis and characterization of granuloma.

• Tuberculosis and Respiratory Diseases
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• v.62 no.5
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• pp.417-420
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• 2007

Sarcoidosis is a multisystemic granulomatous disease with an of unknown etiology, involving bilateral hilar lymphadenopathy, pulmonary, skin and eye lesions. However, involvement of the endocrine system in sarcoidosis is quite rare, and the coexistence of both diseases is extremely unusual. We describe a 60-year-old woman presenting with sarcoidosis and Graves' disease. She was admitted for evaluation of dry cough, dyspnea, palpitation and general weakness. Both thyroid glands were enlarged diffusely. The thyroid function tests showed suppressed serum thyrotropin and an increased thyroid hormone level. The levels of the TSH receptor antibody, anti-thyroglobulin antibody and anti-microsomal antibody were higher than normal. The radionuclide scan($^{131}I$) showed increased iodine uptake. The chest X-ray revealed pulmonary hilar enlargement and high resolution CT showed both hilar lymph nodes enlargement and tiny parenchymal nodules. The transbronchial lung biopsy showed a noncaseating granuloma without necrosis. We report this case of pulmonary sarcoidosis plus Graves' disease with a review of the relevant literatures.