Archive for the ‘Sarcoidosis’ Category


Extensive interstitial pneumonitis was the predominant pathologic finding in this patient with sarcoidosis. Although granulomata were present in the alveolar septa, their sparseness in the pulmonary parenchyma fails to explain the marked degree of interstitial inflammation present. While interstitial pneumonitis has been associated with diseases in which there are altered immune mechanisms, there was no clinical or serologic evidence for an immunologic disorder in this patient. Interstitial pneumonitis in sarcoidosis is infrequently recognized. Katzenstein and Askin state that there is little, if any, interstitial pneumonitis surrounding the granulomata in the lung. Spencer makes no mention of interstitial pneumonitis in his discussion of the histopathologic findings in sarcoidosis. In contrast, Rosen and co-workers reported finding focal, nonspecific interstitial pneumonitis in 62 percent of 128 open lung biopsies performed for presumed sarcoidosis. All biopsies from their series demonstrated granulomata. Based upon their observation that several regions of pneumonitis were organizing into granulomata, Rosen et al suggested that interstitial pneumonitis represented the earliest phase of granuloma development. Our patient differed from their cases in two respects. First, the interstitial pneumonitis was diffuse, and, secondly, the patient had evidence of advanced sarcoidosis, with parenchymal honeycombing and fibrosis. medicines-for-diabetes.com (more…)

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  • Diffuse Interstitial Pneumonitis and Fibrosis in Sarcoidosis

    Feb 9, 2015 Author: Walter Mcneil | Filed under: Sarcoidosis

    Diffuse Interstitial Pneumonitis and Fibrosis in SarcoidosisSarcoidosis is a multisystem disorder of unknown etiology characterized pathologically by the formation of nonnecrotizing granulomata in multiple sites in the body. Although the clinical and radiographic presentation of cases is quite characteristic, a histologic diagnosis is necessary before beginning treatment. Fiberoptic bronchoscopy with trans-bronchial lung biopsies is diagnostic in 60 to 95 percent of cases, depending upon the clinical stage of the disease. The diagnosis is established when non-necrotizing epithelioid granulomata with giant cells are found on biopsy and cultures are negative. While interstitial fibrosis and interstitial pneumonitis have been reported in sarcoidosis, they are usually localized to areas of extensive involvement with active granulomatous disease. We report a case of pulmonary sarcoidosis with extensive interstitial pneumonitis and fibrosis in regions without active granuloma formation.
    Case Report
    A previously healthy 27-year-old black woman presented to the emergency room at the University of Maryland Medical Systems complaining of dyspnea and a nonproductive cough for the past 12 months. Her exercise capacity had diminished to the point of severe dyspnea after walking one block. She recently noted arthralgias, and there was a 5.4-kg (12-lb) weight loss. There was a 14 pack-year history of smoking, but the patient had discontinued smoking cigarettes three months prior to admission. She had previously smoked marijuana for many years and recently began snorting cocaine. There was no occupational history of exposure to toxins.
    Physical examination was remarkable only for the presence of fine end-inspiratory crackles on auscultation and digital clubbing. Admitting laboratory studies revealed a hematocrit reading of 47 percent and a white blood cell count of 6,100/cu mm, with a normal differential. The serum calcium level was 9.6 mg/dl, and the alkaline phosphatase level was 366 units (normal, 95 to 300 units). An arterial blood specimen while breathing room air revealed a pH of 7.39, an arterial carbon dioxide tension of 35 mm Hg, and an arterial oxygen pressure of 72 mm Hg. The chest roentgenogram demonstrated bilateral paratracheal and perihilar lymphadenopathy with a diffuse, reticulonodular parenchymal infiltrate (Fig 1). There was no pleural thickening or effusion. The patient was unable to perform pulmonary function tests due to dyspnea and cough.
    The patient underwent flexible fiberoptic bronchoscopic examination, which showed a cobblestone pattern in the airways but no endobronchial lesions. Microscopic examination of the transbronchial biopsies revealed diffuse interstitial fibrosis and inflammation (Fig 2). No granulomata or giant cells were seen. Pulmonary macrophages and mononuclear cells were found within the alveolar air spaces. These findings were suggestive of a diagnosis of desquamative interstitial pneumonitis; however, because this diagnosis failed to explain the intrathoracic adenopathy, the patient underwent mediastinal lymph node and open lung biopsies.
    Histologic examination of the mediastinal lymph node revealed complete effacement of the normal nodal architecture by nonnecrotizing epithelioid granulomata. The wedge biopsy from the lingula revealed no areas of normal lung. The parenchyma showed extensive interstitial pneumonitis and fibrosis, with reactive alveolar cell hyperplasia in areas not associated with active granuloma formation (Fig 3). There were focal aggregates of acid-fast bacillus-negative non-necrotizing granulomata within the alveolar septa. Peripheral honeycombing with formation of bullae was evident in the subpleura. The patient was discharged on a regimen of 40 mg of prednisone. On follow-up, she was no longer coughing, her exercise tolerance had improved, and she was gaining weight.

    Figure 1. Posteroanterior (left) and lateral (right) chest roentgenograms on admission. Bilateral paratracheal and perihilar adenopathy is present. Reticulonodular infiltrates are present throughout the lung fields.

    Figure 1. Posteroanterior (left) and lateral (right) chest roentgenograms on admission. Bilateral paratracheal and perihilar adenopathy is present. Reticulonodular infiltrates are present throughout the lung fields.

    Figure 2. Transbronchial lung biopsy showing interstital thickening with chronic inflammatory cells. xNote alveolar cell hyperplasia (thick arrow) and mononuclear cells in alveolar spaces (thin arrow) (hematoxylin-eosin, original magnification X900).

    Figure 2. Transbronchial lung biopsy showing interstital thickening with chronic inflammatory cells. xNote alveolar cell hyperplasia (thick arrow) and mononuclear cells in alveolar spaces (thin arrow) (hematoxylin-eosin, original magnification X900).

    Figure 3. Open wedge biopsy from peripheral lung. Note marked interstital fibrosis and alveolar cell hyperplasia. Granulomata are not seen in this field (hematoxylin-eosin, original magnification X570).

    Figure 3. Open wedge biopsy from peripheral lung. Note marked interstital fibrosis and alveolar cell hyperplasia. Granulomata are not seen in this field (hematoxylin-eosin, original magnification X570).

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