Sarcoidosis: An Update for the Primary Care Physician

Oluranti A. Aladesanmi, MD, MPH

Disclosures
In This Article

Clinical Presentation

  • Asymptomatic: hilar lymphadenopathy on routine chest x-ray in 30% to 60% of patients.

  • Pulmonary: 20% to 30% of patients with symptoms; bronchial hyperreactivity in 20% of patients:

    • Cough, dyspnea, wheezing, chest pain -- commonest symptoms;

    • Hemoptysis in < 5% of cases (advanced disease with mycetomas, bronchiectasis, and endobronchial disease); and

    • Rarely, pleural effusions, pleural thickening and calcification, pneumothorax and lymph node calcification.

Extrapulmonary

  • Constitutional: fever, fatigue, malaise, and weight loss in 30% of patients; night sweats occasionally.

African American and Asian Indian Individuals: More Often Symptomatic

  • Lymphatics: palpable peripheral lymphadenopathy in 30% of patients (nontender, discrete, firm, rubbery, and nonadherent):

    • Intrathoracic lymphadenopathy in 75% to 90% of patients; and

    • Splenomegaly, mild and asymptomatic, may cause abdominal discomfort, cytopenias.

  • Skin: 30% of patients:

    • Erythema nodosum, lupus pernio, subcutaneous nodules, plaques, maculopapular rashes, alopecia, abnormal skin pigmentation;

    • Erythema nodosum: common in European, Puerto Rican, and Mexican individuals. Rare in Japanese and African American individuals;

    • Lupus pernio: common in African American women, represents chronic sarcoidosis, indurated plaques, and discoloration of nose, cheeks, lips and ears; nasal mucosa often involved, associated with bone cysts and pulmonary fibrosis; and

    • Chronic sarcoidosis skin lesions: do not cause itch, pain, or ulcerate.

  • Eyes: redness, photophobia, decreased visual acuity in 9% to 25% cases:

    • Anterior uveitis: the commonest eye manifestation, chronic uveitis can cause glaucoma, cataracts, and blindness; and

    • Lacrimal gland involvement can cause keratoconjunctivitis sicca.

  • Cardiac: clinically diagnosed in 5% of cases, found in 20% to 30% of cases at autopsy:

    • Sudden cardiac death, ventricular tachycardia, other arrhythmias, conduction abnormalities, cardiomyopathy, right heart failure due to advanced interstitial lung disease.

  • Hepatic: granulomas in 50% to 80% of cases, palpable hepatomegaly in less than 20% of cases. Mild LFT abnormalities.

  • Musculoskeletal: arthralgia in 25% to 40% of patients; deforming arthritis is rare; knees, elbows, wrists, and small joints of hands and feet are most commonly involved.

  • Neurologic: less than 10% cases:

    • Base of brain predilection, cranial nerve involvement, especially transient unilateral facial nerve paralysis;

    • Diabetes insipidus: hypothalamus and pituitary involvement; and

    • Space occupying lesions, peripheral neuropathy, and neuromuscular involvement occur late with chronic course.

  • Endocrine/renal:

    • Excessive 1,25 dihydroxyvitamin D production by granulomatous tissue causing hypercalcemia (2% to 10% of cases) and hypercalciuria (6% to 30% cases), may lead to nephrocalcinosis, renal calculi, and renal failure.

  • Reproductive:

    • Breast masses, infertility is rare, no direct adverse effect on mother or fetus, may flare up postpartum; and

    • Testicular masses, leading to unnecessary orchiectomies because of suspected malignancy.

Acute Sarcoidosis Syndromes

  • Lofgren's syndrome: erythema nodosum, bilateral hilar/right paratracheal lymphadenopathy, fever, polyarthralgia.

  • Heerfordt's Waldensröm syndrome: fever, uveitis, parotid enlargement, and facial nerve palsy.

Radiographic Staging

  • Stage 0: Normal chest radiograph

  • Stage 1: Bilateral hilar lymphadenopathy

  • Stage 2: Bilateral hilar lymphadenopathy with diffuse pulmonary infiltrates

  • Stage 3: Diffuse pulmonary infiltrates without lymphadenopathy

  • Stage 4: Pulmonary fibrosis

These stages are prognostic, the 5-year mortality being 0% in Stage 1, 11% in Stage 2, 18% in Stage 3, and more than 50% in Stage 4.[14]

Diagnostic Criteria

  • Typical clinical and radiological findings

  • Histologic evidence of noncaseating epitheloid cell granulomas

  • Reasonable exclusion of other granulomatous diseases

Differential Diagnoses[14]


  • Infection

    • Tuberculosis

    • Atypical mycobacterial infections

    • Histoplasmosis and other fungal infections

    • Nocardiosis

    • Actinomycosis

  • Reactions to foreign agents

    • Hypersensitivity pneumonitis

    • Chronic berylium disease

    • Talc lung

  • Vasculitis

    • Wegener's granulomatosis

    • Allergic granulomatosis

    • Lymphomatoid granulomatosis

  • Malignancy (local sarcoid reaction)

    • Lymphoma

    • Carcinoma

  • Other

    • Eosinophilic granuloma

Initial Evaluation of Sarcoidosis Patients[15]


  • Determine extent and severity of disease

    • Chest x-ray

    • Pulmonary function tests spirometry , DLCO

    • Liver enzymes

    • BUN and creatinine, electrolytes and calcium

    • Complete blood count

    • Urinalysis

  • Exclude critical organ involvement

    • EKG

    • Complete ophthalmologic exam

    • Complete neurologic exam

  • Exclude other diseases

    • Occupational and environmental exposure history

    • PPD

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