A Concise Review of Pulmonary Sarcoidosis

Department of Internal Medicine, University of Cincinnati Medical Center, Ohio, USA.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 13). 10/2010; 183(5):573-81. DOI: 10.1164/rccm.201006-0865CI
Source: PubMed


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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    • "However, diagnosing it can be challenging[20]. The gold standard is histopathological proof from biopsy tissue[21,22]. As intraocular tissues are rarely biopsied due to the risk of vision loss, biopsies from nonocular tissues such as lung, skin and peripherial lymph nodes have been taken into account[2,23]. "
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    ABSTRACT: Purpose: To study the impact of soluble IL2 receptor (sIL2R), chest x-ray (CxR), and angiotensin-converting enzyme (ACE) as markers for sarcoidosis in uveitis patients. Design: Retrospective study. Methods: Serum concentrations of sIL2R and ACE were measured in patients with active uveitis. Those with elevated sIL2R and /or ACE values were examined for suspected systemic sarcoidosis. Main outcome measure: Our main outcome parameters were the specificity and sensitivity of sIL2R, CxR and ACE in screening for ocular sarcoidosis. Results: We measured 261 patients with uveitis for sarcoidosis using sIL2R and ACE between January 2008 and November 2011; sarcoidosis was been diagnosed using other tests (e.g. computer tomography, brochoalveolar lavage, biopsy) in 41 of 53 patients with elevated sIL2R values (>639 U/ml) and in one patient with normal sIL2R (582 U/ml). Their mean sIL2R value was 1310 U/ml, extending from 582 to 8659 U/ml. Only 9 patients, however, presented elevated ACE (>82 U/l). Their mean ACE value was 116.4 U/l, ranging from 84.1 to 175.5 U/l. IL2R specificity was 94% with 98% sensitivity. In contrast, ACE had a specificity of 99.5%, but a sensitivity of only 22%; the chest x-ray had a specificity of 100% with 50% sensitivity in detecting sarcoidosis. We observed the entire spectrum of uveitis: sixteen patients suffered from anterior, 8 from intermediate, 16 from posterior, and 2 from panuveitis. Conclusions: An elevated level of soluble IL2R suggests sarcoidosis with uveitis more convincingly than ACE, making sIL2R a more effective marker parameter for sarcoidosis than ACE or chest x-ray in uveitis patients.
    Full-text · Article · Jan 2016 · PLoS ONE
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    • "Some researchers have considered that the development of sarcoidosis is probably the end result of immune responses to various ubiquitously existing environmental triggers, especially in rural settings [20] [29] [30]. Furthermore, there is mounting epidemiological evidence that environmental exposure to certain microbial agents increases the risk of developing sarcoidosis [20] [21] [31] and that mycobacteria and propionibacteria might be likely carriers of the triggering antigen [14] [32]. Nevertheless, the diversity and intensity of microbial stimuli to which we are exposed, especially during early childhood, may also contribute to the disease development later in life by affecting the innate immune system and subsequently causing immune deviation toward an enhanced Th1 response [33e36]. "
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    ABSTRACT: National surveys conducted in Japan between 1960 and 2004 suggest a gradually increasing incidence of sarcoidosis in women >50 years old with increased involvement of the eye, skin, and heart. However, whether this involvement is due to the increased age at diagnosis is still unclear. We aimed here to identify the age-related differences in organ involvement in sarcoidosis in Japan, as well as the historical changes in clinical characteristics and the age-specific distribution of cases at diagnosis. We reviewed 588 consecutive Japanese patients newly diagnosed with sarcoidosis between 1974 and 2012 at Jichi Medical University Hospital. We compared organ involvement between subgroups differentiated by sex and age (<45 years; n = 275; ≥45 years; n = 313) at diagnosis and identified historical changes in the age-specific distribution in 10-year intervals. Younger patients had more common involvement of extrathoracic lymph nodes, parotid/salivary gland, and liver, while older patients had more common involvement of non-lymphatic extrathoracic organs such as the eye, heart, muscle, and kidney. The age at diagnosis has consistently increased over the past four decades. The monophasic distribution in men has tended to become biphasic, and the biphasic distribution in women monophasic. Increasing trends were apparent for hypercalcemia and involvement of the gastrointestinal tract, skin, nervous system, muscle, and kidney. Elderly patients at diagnosis had various extrathoracic involvement including eye, skin, and cardiac lesions. Moreover, the age at diagnosis of sarcoidosis has continued to increase in both sexes, influencing the recent trends in clinical characteristics. Copyright © 2015 Elsevier Ltd. All rights reserved.
    Full-text · Article · Jan 2015 · Respiratory Medicine
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    • "Sarcoidosis is an inflammatory disease, often leading to granuloma formation [1] [2]. Several studies demonstrate that the amounts of inflammatory cytokines, particularly interleukin(IL- ) 10 and IL-12, are elevated in serum and in bronchoalveolar lavage fluid [3] [4] [5] [6]. "
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    ABSTRACT: Background and objectives: Sarcoidosis is an inflammatory disease with increased levels of inflammatory cytokines. Previous studies have shown a relation between the degree of granuloma infiltration and serum cytokine levels, except for interleukin- (IL-) 10. The aim of the study was to further investigate the serum levels of IL-10 in patients with sarcoidosis and relate them to fungal exposure in terms of the amount of fungi in the air of their homes and β-glucan in bronchoalveolar lavage (BAL) fluid. Methods: Patients with sarcoidosis (n = 71) and healthy controls (n = 27) were enrolled. IL-10 was determined in serum. BAL was performed and the amount of β-glucan was measured. Domestic exposure to fungi was determined by measuring airborne β-N-acetylhexosaminidase (NAHA) in the bedrooms. Results: At high levels of fungal exposure (domestic fungal exposure and β-glucan in BAL), serum IL-10 values were lower than at low and intermediate exposure levels. Conclusion: The low serum IL-10 values at high fungal exposure suggest that fungal cell wall agents play a role in granuloma formation in sarcoidosis by inhibiting the secretion of the anti-inflammatory cytokine IL-10.
    Full-text · Article · Aug 2014 · Pulmonary Medicine
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