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Clinical features supportive of a diagnosis of sarcoidosis

Clinical features supportive of a diagnosis of sarcoidosis
  Highly probable Probable
History
  • Löfgren syndrome*
  • Seventh cranial nerve paralysis
  • Treatment-responsive renal failure
  • Treatment-responsive CM or AVNB
  • Spontaneous/inducible VT with no risk factors
Physical
  • Lupus pernio
  • Uveitis
  • Optic neuritis
  • Erythema nodosum
  • Maculopapular, erythematous, or violaceous skin lesions
  • Subcutaneous nodules
  • Scleritis
  • Retinitis
  • Lacrimal gland swelling
  • Granulomatous lesions on direct laryngoscopy
  • Symmetrical parotid enlargement
  • Hepatomegaly/splenomegaly
Imaging
  • Bilateral hilar adenopathy (CXR, CT, and PET)
  • Perilymphatic nodules (chest CT)
  • Gadolinium enhancement on MRI (CNS)
  • Osteolysis, cysts/punched-out lesion, trabecular pattern bone (radiograph, CT, and MRI)
  • Parotid uptake (gallium and PET)
  • Upper lobe or diffuse infiltrates (CXR, CT, and PET)
  • Peribronchial thickening (CT)
  • Two or more enlarged extra thoracic nodes (CT, MRI, and PET)
  • Increased inflammatory activity in heart (MRI, PET, and gallium)
  • Imaging showing enlargement or nodules in liver or spleen (CT, PET, and MRI)
  • Inflammatory lesions in bone (gallium, PET, and MRI)
Other testing
  • Hypercalcemia or hypercalciuria with abnormal vitamin D metabolism
  • Reduced LVEF with no risk factors (echo and MRI)
  • Elevated ACE level testΔ
  • Nephrolithiasis with calcium stone, no vitamin D testing
  • BAL lymphocytosis or elevated CD4:CD8 ratio
  • Alkaline phosphatase greater than three times the upper limit of normal
  • New-onset, third-degree AV block in young or middle-aged adults
CM: cardiomyopathy; AVNB: atrioventricular node block; VT: ventricular tachycardia; CXR: chest radiograph; CT: computed tomography; PET: positron emission tomography; MRI: magnetic resonance imaging; CNS: central nervous system; LVEF: left ventricular ejection fraction; ACE: angiotensin-converting enzyme; BAL: bronchoalveolar lavage; AV: atrioventricular.
* Löfgren syndrome is defined as bilateral hilar adenopathy with erythema nodosum and/or periarticular arthritis.
¶ Abnormal vitamin D metabolism is defined as normal to low parathyroid hormone, normal to elevated 1,25-dihydroxyvitamin D, and normal to low 25-hydroxyvitamin D.
Δ ACE elevated above 50% of the upper limit of normal was considered abnormal.
Reprinted with permission of the American Thoracic Society. Copyright © 2020 American Thoracic Society. All rights reserved. Crouser ED, Maier LA, Wilson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med 2020; 201:e26. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.
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