Chest
Recent Advances in Chest MedicineRecent Advances in Sarcoidosis
Section snippets
Etiology
Progress has been made in elucidating the cause of sarcoidosis. The ACCESS trial collected data on 704 patients with newly diagnosed, biopsy specimen-proven sarcoidosis and control subjects matched by age, sex, race, and geographic area.4 The study identified several environmental exposures modestly associated with sarcoidosis risk (OR, ∼ 1.5).8 The associated exposures included agricultural employment, mold or mildew, musty odors at work, and pesticide-using industries. Tobacco use was
Genetics
Genetic and host factors are involved in the pathogenesis of sarcoidosis (Fig 1).12 Twin studies indicate that monozygotic twins are more often concordant for disease than dizygotic twins.14 In the United States, blacks are more frequently affected by sarcoidosis than other ethnic groups and generally have chronic and more severe disease.12 Familial clustering of sarcoidosis cases has been observed worldwide.15 In ACCESS, subjects were five times more likely than control subjects to report a
Biomarkers of Disease Activity
Investigators continue to search for potential biomarkers of disease activity in sarcoidosis. Chitotriosidase, an enzyme involved in the degradation of chitin, is expressed by activated macrophages. Bargagli and colleagues53 found elevated levels of chitotriosidase in the serum of patients with sarcoidosis compared with control subjects, with > 90% exhibiting increased levels of the marker. Significantly higher levels were observed in patients with active sarcoidosis than in those with inactive
Diagnosis
BAL can be used as an adjunctive measure to support the diagnosis of sarcoidosis by demonstrating a reduced number of CD8 cells and an elevated CD4/CD8 ratio. But the diagnosis of sarcoidosis is best supported by obtaining tissue specimens that show noncaseating granulomas. EBUS-TBNA allows real-time ultrasound localization and aspiration of hilar and mediastinal lymph nodes. Tremblay and colleagues55 compared the diagnostic yield of TBNA using a 19-gauge needle vs EBUS-TNBA in 50 patients with
Cardiac Sarcoidosis
Cardiac sarcoidosis is a potentially sudden and life-threatening manifestation of sarcoidosis. Current studies likely underestimate the true prevalence of disease. Cardiac sarcoidosis is difficult to diagnose. Most patients have minimal or no symptoms, and no gold standard for diagnosis exists. Results from endomyocardial biopsy specimens, which may be difficult to obtain, are positive in < 10% of patients.56 Occasionally, echocardiogram or nuclear stress testing will reveal findings consistent
Therapy
Patients with chronic sarcoidosis often require prolonged treatment. Sustained treatment with even modest doses of systemic corticosteroids may result in disabling side effects. Steroid-sparing agents often are administered to minimize the long-term side effects of systemic corticosteroids.
TNF inhibitors have been investigated for the treatment of sarcoidosis. Utz and colleagues61 assessed the efficacy of etanercept in a preliminary clinical trial of patients with progressive pulmonary
Conclusion
Although the etiology of sarcoidosis remains uncertain, recent studies suggest that mKatG is a pathogenic antigen in sarcoidosis. Much has been learned about the genetic aspects of the disease. HLA gene loci and polymorphisms in transforming growth factor-β and TNF-α strongly influence individual susceptibility to sarcoidosis and clinical phenotype. Novel genes that determine the immunologic features of sarcoidosis have been identified. Reduced numbers of NKT cells may promote an exaggerated
Acknowledgments
Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflict of interest: Dr Morgenthau owns stock in Johnson & Johnson. Dr Iannuzzi has reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
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2019, Seminars in Ultrasound, CT and MRICitation Excerpt :Hemoptysis occurs in as many as 90% of patients with sarcoidosis complicated by mycetomas, and therefore the detection of mycetomas may prompt treatment with antifungal agents.35 Pulmonary hypertension was once thought to be rare but is now considered a relatively common complication of sarcoidosis, although its precise prevalence in this setting is not known.36 Individual series report pulmonary hypertension occurring in 5%-15% of patients with sarcoidosis, and in up to 50% of patients with symptomatic sarcoidosis, with the highest prevalence in patients who are listed for lung transplantation.2
Sarcoidosis-Associated Disability
2018, Sarcoidosis: A Clinician's GuideDiagnostic Pathology: Thoracic
2017, Diagnostic Pathology: ThoracicEfficacy of adalimumab in sarcoidosis patients who developed intolerance to infliximab
2016, Respiratory MedicineCitation Excerpt :In case of treatment failure or serious adverse events with the first-line treatment with glucocorticosteroid, second-line agents are methotrexate (MTX) and azathioprine [2–9]. Finally, third-line therapy consists of monoclonal antibodies targeted specifically at tumor necrosis factor-alpha (TNF-α), a key cytokine in sarcoidosis [10–12]. Of these anti-TNF-α agents, the most extensively studied and most often used therapy is infliximab [13–17].
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