Nasopharyngeal sarcoidosis: a rare involvement

Sarcoidosis is a chronic, multisystemic, non-caseating granulomatous disease of unknown etiology. Nasopharyngeal involvement is very rare in sarcoidosis. The objective of this report was to evaluate a rare involvement of sarcoidosis. This report includes a case of nasopharyngeal sarcoidosis. A 51-year-old female with nasopharyngeal sarcoidosis was treated as sarcoidosis, and she was better. Nasopharyngeal involvement is very rare in sarcoidosis but it must be kept in mind.     

Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics.

Pulmonary hypertension (PH) is a predictor of poor outcome in sarcoidosis. Little is known about the epidemiology of PH in sarcoidosis. The current authors reviewed the records of patients with sarcoidosis listed for lung transplantation in the USA between January 1995 and December 2002. PH was defined as a mean pulmonary artery pressure of >25 mmHg and severe PH as a mean pulmonary artery pressure of > or =40 mmHg. The cohort included 363 patients of whom 73.8% had PH. Neither spirometric testing nor the need for corticosteroids was associated with PH. Subjects with PH required more supplemental oxygen (2.7+/-1.8 L.min(-1) versus 1.6+/-1.4 L.min(-1)). The cardiac index was lower in individuals with PH, whereas the pulmonary capillary wedge pressure was higher. In multivariate analysis, supplemental oxygen remained an independent predictor of PH, whereas the relationship between cardiac index and PH was no longer significant. As a screening test, the need for oxygen had a sensitivity and specificity of 91.8% and 32.6%, respectively. Pulmonary hypertension is common in advanced sarcoidosis. The need for oxygen correlates with pulmonary hypertension. Since pulmonary hypertension is associated with poor outcomes and because simple clinical criteria fail to identify patients with sarcoidosis and pulmonary hypertension, more aggressive screening for this should be considered.     

Pulmonary hypertension in sarcoidosis: a review

Pulmonary hypertension (PH) is a well-recognized complication of sarcoidosis. Patients with sarcoidosis-associated PH (SAPH) have poorer functional status and greater supplemental oxygen requirements than sarcoidosis patients without PH, and are more likely to be listed for lung transplantation. PH is an independent risk factor for mortality in sarcoidosis patients awaiting lung transplantation. The pathophysiology of SAPH is complex, with multiple mechanisms contributing to pathogenesis, including the fibrous destruction of the pulmonary vascular bed, extrinsic compression of the central pulmonary vessels and an intrinsic vasculopathy. Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. Management of SAPH is based on reversal of resting hypoxaemia, treatment of comorbidities and treatment of the underlying sarcoidosis. The use of corticosteroids in SAPH is controversial. Specific PH therapy is not routinely recommended in SAPH as there are no successful placebo-controlled trials, although there is limited data to suggest that endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be useful.     

Postoperative residual pleural spaces: characteristics and natural history

This study was conducted in order to re-define the incidence and natural history of postresectional residual pleural spaces (PRS). From 1997 to 2005, 966 patients who were subjected to less than entire lung resections, were followed and any cases of PRS were recorded. The records of these patients were retrospectively analyzed for age, gender, type of resection, side, apical or basal location, size, PRS wall thickness, empyema as well as for bronchopleural fistula occurence, management, and outcome. Postresectional residual pleural spaces outcome was correlated with space characteristics. A total of 92 cases (9.5%) of PRS were documented which developed frequently ( p < 0.001) after upper lobectomies, malignant disease, at an apical location, and on the right side. Unfavorable outcome was strongly correlated with age > 70 years ( p < 0.001), air leak ( p < 0.001), empyema ( p < 0.001), and thickened pleura ( p < 0.001). Good prognosis of PRS was strongly correlated with male gender, apical location, right side, normal pleura thickness, and small size. Postresectional residual pleural spaces of small size without any associated complications should not prolong hospitalization time.     

Vascular sarcoidosis: a rare cause of pulmonary hypertension

Pulmonary hypertension is an uncommon complication of sarcoidosis and is usually related to the development of end-stage, interstitial fibrosis or honeycomb lung. We describe a patient with sarcoidosis who developed pulmonary hypertension in the absence of significant parenchymal fibrosis. The pulmonary hypertension in this case was caused by granulomatous destruction of arteries and veins, as documented by an open-lung biopsy and autopsy. Clinical features, including pulmonary function tests, are presented which suggested that end-stage interstitial lung disease was not causing the hypertension. Recognition of this complication of sarcoidosis is important if effective therapy is to be found.     

Isolated renal sarcoidosis: a rare presentation of a rare disease treated with infliximab

Sarcoidosis is a multisystem disease characterized by noncaseating granulomatous reaction frequently involving the lymph nodes, lungs, liver, skin, and eyes. Acute renal failure (ARF) as an isolated manifestation of sarcoidosis is rare. We describe a case of sarcoidosis presenting as transient polyarthritis and ARF due to isolated granulomatous infiltration of the renal parenchyma. Renal biopsy showed granulomatous interstitial nephritis with noncaseating granulomas consistent with sarcoidosis. Bacterial, fungal, and mycobacterial infections were excluded. There was no evidence of extrarenal sarcoid involvement. Prednisone of 60 mg daily resulted in significant improvement in renal function. Because of recurrent flares on steroid taper and steroid toxicity, treatment with infliximab, an anti-tumor necrosis factor-α (TNF-α) antibody, was instituted and resulted in stabilization of renal function despite steroid taper. Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis. Corticosteroids are mainstay of therapy. Steroid-dependant or refractory cases may respond to other immunosuppressants including anti-TNF-α agents.     

Pulmonary sarcoidosis: Diagnostic challenges

Sarcoidosis is a multisystemic granulomatosis of unknown cause that predominantly affects the lung and the lymphatic system, especially intrathoracic. The diagnosis relies on the association of a compatible clinical and radiological presentation, the presence of characteristic histopathological lesions (non-necrotizing epithelioid granuloma with giant cells) and the exclusion of other potential causes of granuloma. A meticulous analysis of other clinical manifestations, histopathology and outcome is necessary, particularly in cases with atypical pulmonary presentation (predominant ground glass opacity, excavated consolidation or masses, honeycombing, pleural involvement, or necrotizing sarcoidosis). Severe complications of the pulmonary disease include stage IV sarcoidosis, pulmonary hypertension, bronchial stenosis and pulmonary aspergillosis. In-depth knowledge of pulmonary sarcoidosis is required to ensure the diagnosis of this non-rare disorder of multiple presentation, to identify potentially severe cases and to guide therapeutic decision.     

Pulmonary oncocytoma: a rare case

Oncocytomas are rare tumors with characteristic histological features of large eosinophilic cells with prominent nucleoli and small round nuclei. They commonly occur in the kidney and salivary glands. Their occurrence in the lungs is very rare; fewer than 6 cases being reported so far in the available world literature. We encountered one such case and present the details of the management and its outcome.     

Pulmonary lymphangioleiomyomatosis: a rare presentation

Pulmonary lymphangioleiomyomatosis (LAM) is a rare idiopathic, cystic disease. We report a case of LAM who presented with nonchylous, bilateral pleural effusion and progressive respiratory failure.     

A case of sarcoidosis with multiple nodules showing cavitary formation

A 24-year-old woman was admitted to our hospital for examinations of multiple nodules in bilateral lung fields. Mediastinal lymph node specimen by surgical resection revealed non-caseating epithelioid cell granuloma which was compatible with sarcoidosis. Some nodules showed cavitary formation without any treatment. Bacteriological and cytological studies yielded negative results. This is a rare case of sarcoidosis with multiple nodules showing cavitary formation.     

Pulmonary sarcoidosis in children: a follow-up study.

Progression of pulmonary sarcoidosis in children remains poorly documented. The aim of this work was to gather follow-up information on pulmonary outcomes in children with sarcoidosis and to obtain data of relevance to a discussion of the optimal length and regimen of glucocorticoid therapy. In the present study, the authors experience of pulmonary sarcoidosis in 21 children referred to the paediatric pulmonary department over a 10-yr period is reported with a documented follow-up of at least 4 yr. Evaluation of the disease during the follow-up included analysis of clinical manifestations, chest radiographs, pulmonary function tests with measurements of the vital capacity (VC), dynamic lung compliance (CL,dyn), lung transfer for CO (TL,CO), and arterial blood gases, as well as bronchoalveolar lavage (BAL) with determination of total and differential cell counts. After initial evaluation the decision was a careful observation of four children without therapy. Corticosteroid treatment was initiated in 17 children. Analysis of results indicated that after 6-12 months of treatment most clinical manifestations of the disease and chest radiograph abnormalities disappeared, and beneficial effects on VC and TL,CO were apparent. After 18 months of steroids no benefit on pulmonary function tests could be noticed, with mainly persistence of alterations of CL,dyn. Results of BAL studies documented the presence of an alveolitis with increased lymphocyte populations throughout the follow-up. Relapses were observed in four children during tapering of prednisone; they were not reported after discontinuation of steroid therapy. Taken together data obtained in the presented population can lead to the following suggestions for the management of pulmonary sarcoidosis in children. BAL should be performed at the initial evaluation to document alveolitis; however, nothing seems to be gained from repeating this investigation during follow-up in the absence of specific reasons. Once the decision to initiate glucocorticoid therapy is made, 18 months may be a reasonable treatment duration. Discontinuation of therapy can be decided even if the pulmonary function tests remain abnormal, but the child should then be carefully monitored for a relapse.     

Case of primary cavitary sarcoidosis: exacerbation after 16 years from onset]

A 46-year-old man presented with the complaint of cough, fever and dyspnea on exercise. Sixteen years previously stage II sarcoidosis was diagnosed, with an ocular lesion. So far, he had had no respiratory symptoms and was followed up without medication. Two months before his visit, he had a cough. He gradually developed a fever and felt dyspnea on exercise. Chest X-ray showed an infiltrative shadow with a cavity in the right upper lobe. Bronchofiberscopy was performed and the biopsy specimen from the cavity wall revealed noncaseating epitheloid cell granulomas, suggesting primary cavitary sarcoidosis. Corticosterid therapy was started. His symptoms improved rapidly, and the infiltrative shadow on the right upper lobe decreased within one month. The cavity wall was thin on the following computed tomography. Thirty-two cases of primary cavity sarcoidosis were reported in Japan from 1975-2005. The mean age of the patients was 26.5 years old and the ratio of males to females was 13:3. In most cases, corticosteroid therapy was effective and nine spontaneously improved cases were reported. To determine the indications for steroid therapy, further examination is needed.