Articles
Sarcoidosis: Clinical Presentation, Diagnosis, and Approach to Treatment

https://doi.org/10.1097/MAJ.0b013e31815d8276Get rights and content

ABSTRACT

Sarcoidosis is an idiopathic granulomatous disease. It usually affects the lung but may involve any organ. The diagnosis may be problematic because known causes of granulomatous inflammation must be excluded. Sarcoidosis may remit spontaneously or remain stable. Therefore, therapy is not mandated for the disease. This report reviews the clinical presentation, diagnostic approach, and treatment of sarcoidosis.

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)

  • D.A. Jabs et al.

    Ocular involvement in chronic sarcoidosis

    Am J Ophthalmol

    (1986)
  • O.P. Sharma et al.

    Myocardial sarcoidosis

    Chest

    (1993)
  • Y. Yazaki et al.

    Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone

    Am J Cardiol

    (2001)
  • A. Uemura et al.

    Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies

    Am Heart J

    (1999)
  • A. Nakazawa et al.

    Usefulness of dual 67Ga and 99mTc-sestamibi single-photon-emission CT scanning in the diagnosis of cardiac sarcoidosis

    Chest

    (2004)
  • O. Vignaux et al.

    Clinical significance of myocardial magnetic resonance abnormalities in patients with sarcoidosis: a 1-year follow-up study

    Chest

    (2002)
  • J. Mana et al.

    Lofgren’s syndrome revisited: a study of 186 patients

    Am J Med

    (1999)
  • O.P. Sharma

    Vitamin D, calcium, and sarcoidosis

    Chest

    (1996)
  • J.J. Gisvold et al.

    Sarcoidosis presenting as spiculated breast masses

    Mayo Clin Proc

    (2000)
  • S. Garwood et al.

    Endobronchial ultrasound for the diagnosis of pulmonary sarcoidosis

    Chest

    (2007)
  • R.P. Baughman et al.

    Crackles in interstitial lung disease: comparison of sarcoidosis and fibrosing alveolitis

    Chest

    (1991)
  • Y.P. Kataria et al.

    Immunology of sarcoidosis

    Clin Chest Med

    (1997)
  • M.A. Judson

    The management of sarcoidosis by the primary care physician

    Am J Med

    (2007)
  • M.A. Judson

    An approach to the treatment of pulmonary sarcoidosis with corticosteroids: the six phases of treatment

    Chest

    (1999)
  • J.E. Gottlieb et al.

    Outcome in sarcoidosis: the relationship of relapse to corticosteroid therapy

    Chest

    (1997)
  • E. Jones et al.

    Hydroxychloroquine is effective therapy for control of cutaneous sarcoidal granulomas

    J Am Acad Dermatol

    (1990)
  • P.E. Barre et al.

    Hydroxychloroquine treatment of hypercalcemia in a patient with sarcoidosis undergoing hemodialysis

    Am J Med

    (1987)
  • J.D. Doty et al.

    Treatment of corticosteroid-resistant neurosarcoidosis with a short-course cyclophosphamide regimen

    Chest

    (2003)
  • J.D. Doty et al.

    Treatment of sarcoidosis with infliximab

    Chest

    (2005)
  • R.P. Baughman et al.

    Thalidomide for chronic sarcoidosis

    Chest

    (2002)
  • E. Neville et al.

    Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients

    Q J Med

    (1983)
  • C.T. Huang et al.

    Mortality in sarcoidosis: a changing pattern of the causes of death

    Eur J Respir Dis

    (1981)
  • A.F. Shorr et al.

    Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics

    Eur Respir J

    (2005)
  • M.A. Judson et al.

    Sarcoidosis manifested as peripheral pulmonary infiltrates

    AJR Am J Roentgenol

    (1993)
  • K.B. Highland et al.

    Is there an anatomic explanation for chest pain in patients with pulmonary sarcoidosis?

    South Med J

    (1997)
  • J. Mana et al.

    Clinical factors predicting persistence of activity in sarcoidosis: a multivariate analysis of 193 cases

    Respiration

    (1994)
  • R.H. Winterbauer et al.

    Clinical interpretation of bilateral hilar adenopathy

    Ann Intern Med

    (1973)
  • W.B. Dawson et al.

    High-resolution computed tomography in pulmonary sarcoidosis

    Semin Ultrasound CT MR

    (1990)
  • K. Nishimura et al.

    Pulmonary sarcoidosis: correlation of CT and histopathologic findings

    Radiology

    (1993)
  • G.W. Hunninghake et al.

    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

    (1999)
  • C.J. Bergin et al.

    Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests

    Radiology

    (1989)
  • M.A. Judson

    Extrapulmonary sarcoidosis

    Semin Respir Crit Care Med

    (2007)
  • Cited by (86)

    • Tumor-like neurosarcoidosis: Pearls and pitfalls

      2021, Interdisciplinary Neurosurgery: Advanced Techniques and Case Management
      Citation Excerpt :

      Sarcoidosis is a diffuse, multisystemic granulomatous disease. Lungs and intrathoracic lymph nodes are the most frequently involved sites [8]. The clinical manifestations of neurosarcoidosis are heterogeneous, as granulomatous inflammation may affect any anatomic substrate including the meninges, cranial nerves, brain, spinal cord and peripheral nerves associated with diverse clinical forms of the disease [9].

    • Sialadenitis

      2021, Surgery of the Salivary Glands
    • Sarcoidosis is a rare cause of infertility: A case report

      2020, Urology Case Reports
      Citation Excerpt :

      Moreover, bilateral testicular infarct, connective tissue diseases, and sarcoidosis should be kept in mind as non-neoplastic lesions.3 In general, the diagnosis of sarcoidosis based on clinical, radiological, and histopathological findings in biopsy specimens, and exclusion of all other pathologies.4 In our case, the diagnosis made with an open testicular biopsy along with clinical and radiological findings.

    View all citing articles on Scopus
    View full text