A noninvasive diagnosis score for patients with possible sarcoidosis

Organ system	Findings highly probable for sarcoidosis (3 points)	Findings somewhat probable for sarcoidosis (2 points)
Lungs	Any of the following: CXR: bilateral hilar adenopathy Chest CT: perilymphatic nodules Chest CT: symmetrical hilar/mediastinal adenopathy PET/Gallium-67: mediastinal/hilar enhancement	Any of the following: CXR: diffuse infiltrates CXR: upper lobe fibrosis Chest CT: peribronchial thickening BAL: lymphocytic alveolitis BAL: elevated CD4/CD8 ratio PET/Gallium-67: diffuse parenchymal lung enhancement TBNA: lymphoid aggregates/giant cells
Brain/nerve	Clinical syndrome consistent with granulomatous inflammation of the neurologic system and An abnormal MRI or CSF findings characteristic of neurosarcoidosis	Isolated facial palsy, negative MRI or Clinical syndrome consistent granulomatous inflammation of the neurologic system without characteristic MRI or CSF findings
Nonthoracic lymph node		Multiple enlarged palpable cervical or epitrochlear lymph nodes without B symptoms or Enlarged lymph nodes identified by imaging in at least 2 peripheral or visceral lymph node stations without B symptoms
Kidney		Treatment-responsive renal failure without alternative diagnosis
Heart		Any of the following: Treatment responsive cardiomyopathy or AV-nodal blockade Reduced LVEF in the absence of other clinical risk factors Spontaneous or inducible sustained VT with no other risk factor Mobitz type II or 3rd degree heart block Patchy uptake on dedicated cardiac PET Delayed enhancement on CMR Positive gallium uptake Defect on perfusion scintigraphy or SPECT scan T2 prolongation on CMR
Skin	Lupus pernio	Any of the following: Subcutaneous nodules or plaques Inflammatory papules within a scar or tattoo Violaceous or erythematous annular lesions Violaceous or erythematous macular, papular lesions around the eyes, nose, or mouth
Eyes	Any of the following: Uveitis Optic neuritis Mutton fat keratic precipitates Iris nodules Snowball/string of pearls (pars planitis)	Any of the following: Lacrimal gland swelling Trabecular meshwork nodules Retinitis Scleritis Multiple chorioretinal peripheral lesions Adnexal nodularity Candle wax drippings
Liver		Any of the following: Abdominal imaging demonstrating hepatomegaly Abdominal imaging demonstrating hepatic nodules Alkaline phosphatase >3 times ULN
Bone marrow	PET displaying diffuse uptake
Spleen		Any of the following: Low attenuation nodules on CT PET/gallium-67 uptake in splenic nodules Splenomegaly on imaging or physical examination
Bone/joints	Typical radiographic features (trabecular pattern, osteolysis, cysts/punched out lesions)	Any of the following: Dactylitis Nodular tenosynovitis Positive PET, MRI, or gallium-67 bone imaging
Ear/nose/throat		Granulomatous changes on direct laryngoscopy or Consistent imaging studies (eg, sinonasal erosion, mucoperiosteal thickening, positive PET scan)
Parotid/salivary glands	Positive gallium-67 scan ("Panda sign") or Positive PET scan of the parotid glands	Symmetrical parotitis with syndrome of mumps or Enlarged salivary glands
Muscles		Positive imaging (MRI, Gallium-67) or Palpable muscle masses
Endocrine	Hypercalcemia or hypercalciuria plus all of the following: A normal serum PTH level A normal or increased 1,25-OH dihydroxy vitamin D level A low 25-OH vitamin D level	Nephrolithiasis plus all of the following: A normal serum PTH level A normal or increased 1,25-OH vitamin D level A low 25-OH vitamin D level or Hypercalciuria without serum PTH and 25 and 1,25 vitamin D levels or Nephrolithiasis with calcium stones (without serum PTH and 25 and 1,25 vitamin D levels)

The clinical sarcoidosis diagnosis score (SDS) requires assessing highly probable or somewhat probable findings for sarcoidosis that do not have an alternative explanation. Using the table above, 3 points are awarded for each highly probable finding and 2 for each somewhat probable finding; no additional points are awarded for multiple findings within a single organ system. A tabulated SDS score ≥10 has shown good specificity (>99%) for the diagnosis of sarcoidosis and may obviate the need for invasive biopsy in some cases.^[1]

For additional details, refer to UpToDate topics on the diagnosis of sarcoidosis.

AV: atrioventricular; BAL: bronchoalveolar lavage; CMR: cardiac magnetic resonance imaging; CSF: cerebrospinal fluid; CT: computed tomography; CXR: chest radiograph; LVEF: left ventricular ejection fraction; MRI: magnetic resonance imaging; PET: positron emission tomography; PTH: parathyroid hormone; SPECT: single-photon emission computed tomography; TBNA: transbronchial needle aspirate; ULN: upper limit of normal; VT: ventricular tachycardia.

Reference:

Jeny F, Vucinic V, Zhou Y, et al. Validation of the Darcoidosis Diagnostic Score in a multicontinental study. Ann Am Thorac Soc 2023; 20:371.

Adapted with permission from: Judson MA, Costabel U, Drent M, et al. The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31:19.

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A noninvasive diagnosis score for patients with possible sarcoidosis