Organ systemPrevalence (% of patients)Clinical manifestationsDiagnostic workup
Mediastinal lymph nodes95 to 98Hilar and mediastinal lymphadenopathyChest radiography, chest CT, endoscopic ultrasonography with needle aspiration (endobronchial or esophageal), gallium scan, 18F-FDG PET (in select patients)
Lungs> 90Cough, dyspneaChest radiography, chest CT (may be necessary)
Hilar and mediastinal lymphadenopathyChest radiography, chest CT, endoscopic ultrasonography with needle aspiration (endobronchial or esophageal), gallium scan, 18F-FDG PET (in select patients)
Pulmonary hypertensionBrain natriuretic peptide, six-minute walk test, echocardiography, right heart catheterization
Interstitial lung disease and pulmonary fibrosisChest radiography, chest CT, bronchoscopy, surgical lung biopsy (if needed)
Liver50 to 80Mostly asymptomatic, mild elevation in liver function testsLiver biopsy if significant laboratory abnormalities
Spleen40 to 80Splenomegaly, rarely pain or cytopeniasAbdominal ultrasonography and abdominal CT
Eyes20 to 50Uveitis, retinal vascular changes, conjunctival nodules, lacrimal gland enlargementOphthalmologic evaluation, lacrimal gland biopsy (if necessary), gallium scan (in select patients)
Musculoskeletal25 to 39Proximal muscle weakness, myalgias, intramuscular nodulesCreatine kinase, MRI, 18F-FDG PET, possible muscle biopsy
Peripheral lymphadenopathy30Peripheral lymph nodes, most commonly cervical and supraclavicular nodesBiopsy of most accessible and safest site, if needed to clarify diagnosis
Hematologic4 to 40Anemia, leukopeniaComplete blood count with differential, bone marrow biopsy in select patients
Skin25Papules, nodules, plaques, erythema nodosum, lupus pernioSkin biopsy as needed, except for erythema nodosum and lupus pernio, which can usually be diagnosed clinically
Nervous system10Cranial nerve palsiesBrain MRI
Optic neuritisOphthalmologic evaluation
HypopituitarismHormonal studies
Cognitive dysfunctionBrain MRI, cerebrospinal fluid studies
Small fiber neuropathyElectromyography, nerve conduction studies
Heart5Conduction abnormalities, arrhythmias (including ventricular tachycardia and ventricular fibrillation), congestive heart failure, sudden deathElectrocardiography, echocardiography, Holter monitoring (depending on symptoms), cardiac MRI, 18F-FDG PET, thallium scan (in select patients)
Hypercalcemia2 to 10Nephrocalcinosis, renal stones, and renal failureBiopsies, renal ultrasonography and CT urography
Parotid glands< 6Isolated or associated with Heerfordt syndrome (uveoparotid fever)Biopsy, gallium scan (in select patients)