Table 1.
Clinical Features Supportive of a Diagnosis of Sarcoidosis
| Highly Probable | Probable | |
|---|---|---|
| History | Löfgren’s syndrome* | Seventh cranial nerve paralysis |
| Treatment-responsive renal failure | ||
| Treatment-responsive CM or AVNB | ||
| Spontaneous/inducible VT with no risk factors | ||
| Physical | Lupus pernio | Maculopapular, erythematous, or violaceous skin lesions |
| Uveitis | Subcutaneous nodules | |
| Optic neuritis | Scleritis | |
| Erythema nodosum | Retinitis | |
| Lacrimal gland swelling | ||
| Granulomatous lesions on direct laryngoscopy | ||
| Symmetrical parotid enlargement | ||
| Hepato-/splenomegaly | ||
| Imaging | Bilateral hilar adenopathy (CXR, CT, and PET) | Upper lobe or diffuse infiltrates (CXR, CT, and PET) |
| Perilymphatic nodules (chest CT) | Peribronchial thickening (CT) | |
| Gadolinium enhancement on MRI (CNS) | Two or more enlarged extra thoracic nodes (CT, MRI, and PET) | |
| Osteolysis, cysts/punched-out lesion, trabecular pattern bone (X-ray, CT, and MRI) | Increased inflammatory activity in heart (MRI, PET, and gallium) | |
| Parotid uptake (gallium and PET) | 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 test‡ | ||
| 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 |
Definition of abbreviations: ACE = angiotensin-converting enzyme; AV = atrioventricular; AVNB = atrioventricular node block; CM = cardiomyopathy; CNS = central nervous system; CT = computed tomography; CXR = chest X-ray; LVEF = left ventricular ejection fraction; MRI = magnetic resonance imaging; PET = positron emission tomography; VT = ventricular tachycardia.
Löfgren’s 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.
ACE elevated above 50% of the upper limit of normal was considered abnormal.