ArticlesSarcoidosis: Clinical Presentation, Diagnosis, and Approach to Treatment
Section snippets
Natural Course
The natural course of pulmonary sarcoidosis is highly variable. Up to 60% of patients are asymptomatic such that pulmonary sarcoidosis is detected by an incidental finding on a chest radiograph.1 Radiographic abnormalities of patients with pulmonary sarcoidosis resolve, improve, or stabilize in 60% to 90% of cases.1., 2. Radiographic resolution is more frequent with hilar adenopathy alone on the initial chest radiograph (radiographic stage I) than with parenchymal infiltration (radiographic
Extrapulmonary Involvement
Sarcoidosis may affect any organ in the body, and extrapulmonary manifestations of the disease are often of major clinical importance. The clinician must be cognizant of the manifestations of sarcoidosis in all organs in order to deliver optimal care. The expertise of various subspecialists should be requested when appropriate. This section will outline the various extrapulmonary manifestations of the disease. Because of space limitations, this topic can only be briefly discussed. The
Diagnosis
The diagnosis of sarcoidosis requires compatible clinical findings, histologic demonstration of noncaseating granulomas, and exclusion of alternative diseases.16 Mycobacterial and fungal diseases must always be considered capable of producing a similar histologic and clinical picture. Therefore stains and cultures for fungi and mycobacteria should always be obtained when the diagnosis of sarcoidosis is considered.
Because sarcoidosis is a diagnosis of exclusion (granulomatous inflammation of
Initial Evaluation
Table 4 lists the recommended initial evaluation of a patient with diagnosed sarcoidosis.16 A medical history should be obtained to consider the exposures that may explain an alternative diagnosis such as tuberculosis, fungal diseases, chronic beryllium disease, and hypersensitivity pneumonitis (eg, significant bird exposure). A physical examination often reveals a normal chest examination66 and is most useful to detect signs of extrapulmonary sarcoidosis such as skin lesions or red eyes that
Treatment
Therapy is not mandated for sarcoidosis because the disease may remit spontaneously. Therapy is indicated for potentially dangerous disease including cardiac sarcoidosis, neurosarcoidosis, hypercalcemia unresponsive to dietary measures, any ocular involvement, and other life-threatening disease.68 Therapy should also be considered when the disease is progressive. In general, asymptomatic patients should not be treated with the exception of those with involvement of the eye and possibly the
Summary
Sarcoidosis is a granulomatous disease that usually affects the lungs but may affect any organ. The diagnosis is one of exclusion and requires elimination of all granulomatous diseases of known cause. All patients with sarcoidosis should be screened for disease in other organs, especially the lungs, eyes, and heart. Treatment of sarcoidosis is not mandated. Corticosteroids are the drug of choice in most instances.
References (84)
- et al.
Pulmonary sarcoidosis
Clin Chest Med
(1997) - et al.
Familial sarcoidosis in Finland and Hokkaido, Japan: a comparative study
Respir Med
(1999) - et al.
Endobronchial biopsy for sarcoidosis: a prospective study
Chest
(2001) - et al.
Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis
Chest
(2006) - et al.
Mediastinoscopy in patients with presumptive stage I sarcoidosis: a risk/benefit, cost/benefit analysis
Chest
(1998) - et al.
Course and prognosis of sarcoidosis
Am J Med
(1960) - et al.
Use of pulmonary function tests in the management of sarcoidosis
Chest
(1980) - et al.
Respiratory muscle strength, lung function, and dyspnea in patients with sarcoidosis
Chest
(2001) - et al.
Predicting mortality in patients with sarcoidosis awaiting lung transplantation
Chest
(2003) - et al.
Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients
Respir Med
(2003)
Ocular involvement in chronic sarcoidosis
Am J Ophthalmol
Myocardial sarcoidosis
Chest
Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone
Am J Cardiol
Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies
Am Heart J
Usefulness of dual 67Ga and 99mTc-sestamibi single-photon-emission CT scanning in the diagnosis of cardiac sarcoidosis
Chest
Clinical significance of myocardial magnetic resonance abnormalities in patients with sarcoidosis: a 1-year follow-up study
Chest
Lofgren’s syndrome revisited: a study of 186 patients
Am J Med
Vitamin D, calcium, and sarcoidosis
Chest
Sarcoidosis presenting as spiculated breast masses
Mayo Clin Proc
Endobronchial ultrasound for the diagnosis of pulmonary sarcoidosis
Chest
Crackles in interstitial lung disease: comparison of sarcoidosis and fibrosing alveolitis
Chest
Immunology of sarcoidosis
Clin Chest Med
The management of sarcoidosis by the primary care physician
Am J Med
An approach to the treatment of pulmonary sarcoidosis with corticosteroids: the six phases of treatment
Chest
Outcome in sarcoidosis: the relationship of relapse to corticosteroid therapy
Chest
Hydroxychloroquine is effective therapy for control of cutaneous sarcoidal granulomas
J Am Acad Dermatol
Hydroxychloroquine treatment of hypercalcemia in a patient with sarcoidosis undergoing hemodialysis
Am J Med
Treatment of corticosteroid-resistant neurosarcoidosis with a short-course cyclophosphamide regimen
Chest
Treatment of sarcoidosis with infliximab
Chest
Thalidomide for chronic sarcoidosis
Chest
Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients
Q J Med
Mortality in sarcoidosis: a changing pattern of the causes of death
Eur J Respir Dis
Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics
Eur Respir J
Sarcoidosis manifested as peripheral pulmonary infiltrates
AJR Am J Roentgenol
Is there an anatomic explanation for chest pain in patients with pulmonary sarcoidosis?
South Med J
Clinical factors predicting persistence of activity in sarcoidosis: a multivariate analysis of 193 cases
Respiration
Clinical interpretation of bilateral hilar adenopathy
Ann Intern Med
High-resolution computed tomography in pulmonary sarcoidosis
Semin Ultrasound CT MR
Pulmonary sarcoidosis: correlation of CT and histopathologic findings
Radiology
ATS/ERS/WASOG statement on sarcoidosis: American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders
Sarcoidosis Vasc Diffuse Lung Dis
Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests
Radiology
Extrapulmonary sarcoidosis
Semin Respir Crit Care Med
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