A Concise Review of Pulmonary Sarcoidosis

Article (PDF Available)inAmerican Journal of Respiratory and Critical Care Medicine 183(5):573-81 · October 2010with42 Reads
DOI: 10.1164/rccm.201006-0865CI · Source: PubMed
Abstract
This is an update on sarcoidosis, focusing on etiology, diagnosis, and treatment. In the area of etiopathogenesis, we now have a better understanding of the immune response that leads to the disease as well as genetic factors that modify both the risk for the disease and its clinical outcome. Several groups have also identified possible agents as a cause for sarcoidosis. Although none of these potential causes has been definitely confirmed, there is increasing evidence to support that one or more infectious agents may cause sarcoidosis, although this organism may no longer be viable in the patient. The diagnosis of sarcoidosis has been significantly aided by new technology. This includes the endobronchial ultrasound, which has been shown to increase the yield of needle aspiration of mediastinal and hilar lymph nodes. The positive emission tomography scan has proven useful for selecting possible biopsy sites by identifying organ involvement not appreciated by routine methodology. It has also helped in assessing cardiac involvement. The biologic agents, such as the anti-tumor necrosis factor antibodies, have changed the approach to refractory sarcoidosis. There is increasing evidence that the clinician can identify which patient is most likely to benefit from such therapy. As new and more potent antiinflammatory agents have been developed, it is clear that there are other factors that burden the patient with sarcoidosis, including fatigue and sarcoidosis-associated pulmonary hypertension. There have been several recent studies demonstrating treatment options for these problems.

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    • "We demonstrated previously that after in vitro stimulation of PBMCs with FCWAs alone, the secretion of TNF-?, IL- 6, IL-10 and IL-12 was higher in patients with sarcoidosis compared to healthy subjects [20]. These cytokines have all been implicated in the immunopathogenesis of sarcoidosis [1,[23][24][25]. Since FCWAs and LPS might have a synergistic effect on the immune responses of patients with sarcoidosis, we investigated in the present study the in vitro synthesis of inflammatory cytokines in PBMCs after co-stimulation with FCWAs and LPS. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Composition of organic dust is very complex, involving particles of microbial, animal and plant origin. Several environmental exposure studies associate microbial cell wall agents in organic dust with various respiratory symptoms and diseases. The aim of the present study was to investigate the in vitro effects of the co-exposure of fungal cell wall agents (FCWAs) and bacterial lipopolysaccharide (LPS) on inflammatory immune responses of peripheral blood mononuclear cells (PBMCs) from patients with pulmonary sarcoidosis. MethodsPBMCs from 22 patients with pulmonary sarcoidosis and 20 healthy subjects were isolated and stimulated in vitro with FCWAs (soluble and particulate (1???3)-?-D-glucan, zymosan and chitosan) and/or LPS. Subsequently, cytokines were measured by ELISA and the mRNA expression of dectin-1, toll-like receptor 2 (TLR2), TLR4 and mannose receptor (MR) was analysed by real-time RT-PCR. ResultsPatients with sarcoidosis had a significantly higher secretion of inflammatory cytokines tumour necrosis factor-alpha (TNF-?), interleukin-6 (IL-6), IL-10 and IL-12 (1.7-fold, 2.0-fold, 2.2-fold, and 2.8-fold, respectively; all p?<?0.05) after in vitro co-stimulation of PBMCs with FCWAs and LPS. We showed that PBMCs from patients with sarcoidosis had a higher baseline mRNA expression of dectin-1, TLR2, TLR4 and MR (6-fold, 11-fold, 18-fold, and 4-fold, respectively). Furthermore, we found a reduced expression of dectin-1, TLR2 and TLR4 after stimulation with FCWAs and/or LPS, although the reduction was significantly weaker in patients than in healthy subjects. Conclusions In conclusion, co-stimulation with FCWAs and LPS of PBMC from patients with sarcoidosis caused a weaker reduction of dectin-1, TLR2, TLR4 receptors expression, which could increase the sensitivity of PBMCs, leading to excessive inflammatory cytokine responses and result in the development or progression of pulmonary sarcoidosis.
    Full-text · Article · Dec 2016
    • "Sarcoidosis is an idiopathic granulomatous disease that can affect every organ system, most commonly the lungs, which are involved in over 90% of cases.[1, 2] There are numerous clinical manifestations of pulmonary sarcoidosis, including pulmonary nodules, fibrotic lung disease, airway hyperreactivity, mucosal nodules and plaques, bronchostenosis, and bronchiectasis.[3] The most common cause of bronchiectasis in sarcoidosis is traction bronchiectasis and has been reported in up to 40% of patie"
    [Show abstract] [Hide abstract] ABSTRACT: Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa. Endobronchial biopsies and lymphocyte subset analysis of bronchoalveolar lavage fluid were consistent with a diagnosis of sarcoidosis. We believe endobronchial sarcoidosis should be included in the differential diagnosis of patients presenting with bronchiectasis.
    Full-text · Article · Nov 2016
    • "Serum ACE and vitamin D levels are often elevated. Biopsy shows non-caseating granulomas, but this is not a pathognomonic finding; other entities such as rheumatoid arthritis, foreign-body reactions (including inhalational diseases) and fungal infection must be excluded [1, 2]. Although musculoskeletal complaints are commonly associated with sarcoidosis, radiographically apparent bone disease is uncommon and is rarely seen at presentation. "
    [Show abstract] [Hide abstract] ABSTRACT: We report a case of fulminant sarcoidosis in a 28-year-old man presenting with skin nodules, multifocal small and large joint arthralgias, and blurred vision. Characteristic bone, soft tissue, articular, and CNS findings were evident on multimodality imaging. Bony abnormalities included near-complete destruction of a distal phalanx, “lace-like” lucent lesions, erosive arthritis, lytic lesions with and without sclerotic margins, and bone marrow replacement visible only on MRI. The extent of bony disease at time of presentation was unusual. We review the widely varying reported prevalence of imaging findings of bony sarcoidosis in the literature, and discuss reasons for this variability. We found that musculoskeletal findings at US and MRI were less specific than radiographic and CT findings, but were useful in quantifying extent of disease.
    Article · Sep 2016
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