Pulmonary Sarcoidosis

Ct, Huang; Ae, Heurich; Rosen, Yale; Moon, Sanggiu; Ha, Lyons

doi:10.1159/000194046

Cited by 52 publications

(3 citation statements)

References 11 publications

(20 reference statements)

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“…Death caused by sarcoidosis in Western countries is most often the result of advanced pulmonary disease with cardiopulmonary failure, whereas deaths from cardiac sarcoidosis in Japan far outnumber those from pulmonary sarcoidosis. 73 Data from three autopsy studies of patients with sarcoidosis [73][74][75] (Tables 1-3) in the United States and Japan show a high percentage of cases with cardiac involvement as the cause of death (Tables 1 and 2). In approximately one third of the cases death was due to a cause other than sarcoidosis (Table 1).…”

Section: Amyloidosismentioning

confidence: 99%

Pathology of Sarcoidosis

Rosen

2007

Semin Respir Crit Care Med

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164

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The role of pathology in the diagnosis of sarcoidosis is identification of granulomas in tissue specimens and performance of studies to exclude known causes of granulomatous inflammation. The granulomas of sarcoidosis are nonspecific lesions that, by themselves and in the absence of an identifiable etiologic agent, are not diagnostic of sarcoidosis or any other specific disease. Among the diseases to be excluded are mycobacterial, fungal, and parasitic infections, chronic beryllium disease and other pneumoconiosis, hypersensitivity pneumonitis, and Wegener's granulomatosis. Even after extensive workup a substantial number of granulomas will remain unclassified. Not every disease that features nonnecrotizing granulomas of undetermined etiology is sarcoidosis. The granulomas of sarcoidosis may exhibit focal necrosis of minimal amount. In cases with granulomas that exhibit a greater degree of necrosis an infectious or other nonsarcoid etiology should be strongly suspected. Strict clinical, radiological, and pathological criteria must be used for diagnosis. In cases that exhibit necrotizing granulomas with more than minimal, focal necrosis, extrathoracic involvement only, and/or incompatible clinical and radiological findings, the diagnosis of sarcoidosis should be approached with great caution. The diagnosis is most secure when compatible clinical and radiological findings are supported by the demonstration of microorganism-negative, nonnecrotizing granulomas in a biopsy specimen accompanied by biopsy evidence or strong clinical evidence of multisystem involvement, and negative cultures for bacteria, mycobacteria, and fungi. A positive Kveim-Siltzbach test provides strong support for the diagnosis of sarcoidosis.

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Section: Amyloidosismentioning

confidence: 99%

Pathology of Sarcoidosis

Rosen

2007

Semin Respir Crit Care Med

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164

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“…In a cohort of 1090 patients with sarcoidosis who were followed up for 17 years, there were 41 deaths, of which 28 (68%) were due to sarcoidosis, primarily from advanced pulmonary disease . In another study of 479 patients with sarcoidosis followed up for 7 years, 22 died (5%) .…”

Section: Prognosis and Clinical Course Of Sarcoidosismentioning

confidence: 99%

Diagnosis and Treatment of Pulmonary Sarcoidosis

Belperio

Shaikh

Abtin

et al. 2022

JAMA

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ImportanceSarcoidosis is an inflammatory granulomatous disease of unknown cause that affects an estimated 2 to 160 people per 100 000 worldwide and can involve virtually any organ. Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease.ObservationAmong patients with pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. However, lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years. Overall, the mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period. Worldwide, more than 60% of deaths from sarcoidosis are due to pulmonary involvement; however, more than 70% of deaths from sarcoidosis are due to cardiac involvement in Japan. Up to 70% of patients with advanced pulmonary sarcoidosis develop precapillary pulmonary hypertension, which is associated with a 5-year mortality rate of approximately 40%. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. Although optimal doses of oral glucocorticoids for pulmonary sarcoidosis are unknown, oral prednisone typically starting at a dose of 20 mg/d to 40 mg/d for 2 to 6 weeks is recommended for patients who are symptomatic (cough, dyspnea, and chest pain) and have parenchymal infiltrates and abnormal pulmonary function test results. Oral glucocorticoids can be tapered over 6 to 18 months if symptoms, pulmonary function test results, and radiographs improve. Prolonged use of oral glucocorticoids may be required to control symptoms and stabilize disease. Patients without adequate improvement while receiving a dose of prednisone of 10 mg/d or greater or those with adverse effects due to glucocorticoids may be prescribed immunosuppressive agents, such as methotrexate, azathioprine, or an anti–tumor necrosis factor medication, either alone or with glucocorticoids combined with appropriate microbial prophylaxis for Pneumocystis jiroveci and herpes zoster. Effective treatments are not available for advanced fibrocystic pulmonary disease.Conclusions and RelevanceSarcoidosis has a mortality rate of approximately 7% within a 5-year follow-up period. More than 10% of patients with pulmonary sarcoidosis develop progressive disease and more than 60% of deaths are due to advanced pulmonary sarcoidosis. Oral glucocorticoids with or without another immunosuppressive agent are the first-line therapy for symptomatic patients with abnormal pulmonary function test results and lung infiltrates. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues.

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“…Cardiac sarcoidosis is the leading or second leading cause of death from the disease in most series [13][14][15]. In addition, unlike other sarcoidosis organ involvement, life-threatening manifestations of cardiac involvement may present suddenly [16,17].…”

Section: The Treatment Of Cardiac Sarcoidosismentioning

confidence: 99%

The treatment of sarcoidosis: translating the European respiratory guidelines into clinical practice

Judson¹

2022

Current Opinion in Pulmonary Medicine

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Purpose of reviewRecently, the European Respiratory Society (ERS) developed new international guidelines for the treatment of sarcoidosis. This manuscript attempts to distill the ERS Sarcoidosis Treatment Guidelines to a manageable format that can be easily used by practitioners.Recent findingsThe ERS Sarcoidosis Treatment Guidelines addressed the treatment of pulmonary, skin, cardiac, neurologic, and sarcoidosis-associated fatigue. Therapeutic drug dosing and treatment algorithms for these conditions were also addressed. Glucocorticoids were the initial recommended treatment for these conditions except for sarcoidosis-associated fatigue where a pulmonary exercise program or a neurostimulant was initially suggested. Because of the risk of glucocorticoid side-effects, the Guidelines recommended early consideration of glucocorticoid-sparing therapy including certain antimetabolites and two specific tumor necrosis alpha antagonists: infliximab and adalimumab.SummaryThe ERS Sarcoidosis Treatment Guidelines used a rigorous GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology to update treatment recommendations for this condition. This manuscript summarizes the Guideline findings in practical terms for clinicians. Suggested algorithms and treatment dosing recommendations are provided.

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Pulmonary Sarcoidosis

Cited by 52 publications

References 11 publications

Pathology of Sarcoidosis

Pathology of Sarcoidosis

Diagnosis and Treatment of Pulmonary Sarcoidosis

The treatment of sarcoidosis: translating the European respiratory guidelines into clinical practice

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