Sarcoidosis Clinical Presentation

Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD more...

Updated: Jul 29, 2011

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History
Physical
Causes
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History

Presentation depends on the extent and severity of the organ involved. Approximately 5% of cases are asymptomatic and incidentally detected by CXR. Systemic complaints, fever, anorexia, and arthralgias occur in 45% of cases. Pulmonary, dyspnea on exertion, cough, chest pain, and hemoptysis (rare) occur in 50% of cases.

Löfgren syndrome symptoms consist of fever, bilateral hilar lymphadenopathy (BHL), and polyarthralgias. This presentation is associated with an excellent prognosis. Although common in Scandinavian patients, it is uncommon in African American and Japanese patients.

Pulmonary findings

They are usually normal but may be significant for crackles. Exertional oxygen desaturation may also be found.

CXR staging system

Stage 0 is normal CXR findings.

Stage I is BHL. See the image below.

Stage I sarcoidosis.

Stage II is BHL and infiltrates. See the image below.

Stage II sarcoidosis.

Stage III is infiltrates alone. See the image below.

Stage III sarcoidosis.

Stage IV is fibrosis.

Dermatological manifestations

Erythema nodosum may occur. A lower extremity panniculitis with painful, erythematous nodules. Associated with Löfgren syndrome but may be seen in other conditions

Lupus pernio is the most specific associated cutaneous lesion.

Violaceous rash is often seen on the cheeks or nose.

Osseous involvement may be present.

Maculopapular plaques are possible.

Ocular manifestations

Anterior or posterior granulomatous uveitis is most frequent.

Conjunctival lesions and scleral plaques may also be noted.

Ocular involvement may lead to blindness if untreated.

Cardiac manifestations

Heart failure from cardiomyopathy rarely occurs.

Heart block and sudden death may occur.

Approximately 25% of patients may have NCGs at autopsy, but fewer than 5% have clinical cardiac disease.

Okada et al reported on cardiac infiltration associated with a novel heterogenous mutation (G481D in CARD15) in early-onset sarcoidosis.^[6]

Neurologic manifestations (rare)

Cranial nerve palsies and hypothalamic/pituitary dysfunction may occur.

Lymphocytic meningitis is the most common neurologic manifestation.

Causes

The cause of the disease is not known; however, both genetic and environmental factors seem to play a role. As yet, no bacterial, fungal, or viral antigen has been consistently isolated from the sarcoidosis lesions. Sarcoidosis is neither a malignant nor an autoimmune disease. An ongoing National Institutes of Health (NIH)–supported study is investigating genetic and acquired causes of sarcoidosis. Additionally, Sverrild et al reported from a twin study that genetic factors play an important role in an individual’s susceptibility to sarcoidosis.^[7]

The following have been suggested as possible candidates that might play a role in causing sarcoidosis. Mycobacteria, such as Mycobacterium tuberculosis, and atypical pathogens have been suggested. Fungi and viruses, particularly Mycoplasma, Chlamydia, and Epstein-Barr virus, have been unconvincingly implicated.

Proceed to Differential Diagnoses

Contributor Information and Disclosures

Author

Nader Kamangar, MD, FACP, FCCP, FCCM Associate Professor of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of California, Los Angeles, David Geffen School of Medicine, Olive View-UCLA Medical Center; Associate Program Director, Pulmonary and Critical Care Multi-Campus Fellowship Program, Cedars-Sinai/West Los Angeles Veterans Affairs/Los Angeles Kaiser Permanente/Olive View-UCLA Medical Center; Site Director, Pulmonary/Critical Care Fellowship Program, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: American Academy of Sleep Medicine, American Association of Bronchology, American College of Chest Physicians, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, California Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Andrew F Shorr, MD, MPH Assistant Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, Uniformed Services University of the Health Sciences

Andrew F Shorr, MD, MPH is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP Professor of Genomics and Personalized Medicine Research, Internal Medicine, and Pediatrics, Associate Director, Center for Genomics and Personalized Medicine Research, Director of Research, Section on Pulmonary, Critical Care, Allergy and Immunologic Diseases, Wake Forest University School of Medicine

Stephen P Peters, MD, PhD, FACP, FAAAAI, FCCP, FCPP is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society, and Sigma Xi

Disclosure: See below for list of all activities None None

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H Professor, Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Southern California Keck School of Medicine

Om Prakash Sharma, MD, FRCP, FCCP, DTM&H is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Osler Society, American Thoracic Society, New York Academy of Medicine, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Timothy D Rice, MD Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society

Disclosure: Nothing to disclose.

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