DESCRIPTION 
- Chronic, multisystem disorder characterized by local accumulation of T lymphocytes and mononuclear phagocytes forming noncaseating epithelioid granulomas
- Symptoms mainly due to organ dysfunction due to disruption of local tissue architecture:
- Predominance of lung symptoms
- ACE and Vitamin D levels may be increased due to secretion from granulomatous tissue
- Prevalence 10–20/100,000 in US and Europe
- Affects almost all races and geographic locations
- Symptoms typically begin in patients 10–40 yr of age
- 2.4% lifetime risk to blacks in US, relative to whites at 0.85%
ETIOLOGY
Unclear, but appears to be an overly robust cell-mediated immune response to unidentified self- or nonself antigen(s)
[Outline]
SIGNS AND SYMPTOMS
History
- Constitutional:
- Skin (25% patients):
- Cardiac/respiratory (most patients):
- Neurologic:
- Ocular (20% patients):
- Renal:
- Musculoskeletal:
Physical Exam
- Constitutional:
- Skin:
- EENT:
- Uveitis
- Keratoconjunctivitis
- Parotid gland enlargement
- Neurologic:
- Nerve palsy (usually CN VII)
- Respiratory:
- Cardiac (~5% patients):
- Dysrhythmias, conduction abnormalities, AV block
- CHF (due to restrictive cardiomyopathy)
- Murmurs (due to papillary muscle dysfunction)
- Renal:
- Musculoskeletal:
- Löfgren syndrome:
- Bilateral hilar adenopathy
- Erythema nodosum
- ±Polyarthralgias
- Heerfordt–Waldenström syndrome:
- Fever
- Uveitis
- Parotid gland enlargement
- ±CN VII palsy
Pediatric Considerations
- Children < 4 yr old classically present with triad of rash, uveitis, and arthritis.
- Children ≥4 yr old present similarly to adults.
ESSENTIAL WORKUP
- Physical exam with emphasis on lung, skin, eye, heart, and musculoskeletal
- Pulse oximetry/ABG
- ECG (dysrhythmias, conduction delays)
- Slit-lamp eye exam
DIAGNOSIS TESTS & INTERPRETATION
Lab
- Serum ACE elevated in 75% cases
- Basic chemistry panel
- LFTs: Mild, usually asymptomatic, mainly elevated alk phos but possible mild elevation transaminases
- Serum calcium: Hypercalcemia due to excessive vitamin D
- UA: Hypercalciuria
- Hypergammaglobulinemia
- CSF analysis: Lymphocyte predominance, elevated ACE level
Imaging
Chest radiograph (abnormal in 90% sarcoid patients)—reason for frequent incidental diagnosis:
- Type 1: Bilateral hilar lymphadenopathy
- Type 2: Lymphadenopathy and parenchymal lung changes (reticular opacities)
- Type 3: Parenchymal lung changes without hilar lymphadenopathy
- Type 4: Reticular opacities, pulmonary fibrosis; particularly in upper lobes
- Radiotracer scans may identify granulomatous disease but is nonspecific
Diagnostic Procedures/Surgery
- Biopsy:
- Bronchoscopy and bronchoalveolar lavage
- Skin lesions if feasible
- Kveim–Siltzbach test:
- Subcutaneous injection of antigen with subsequent spleen biopsy
- Rarely used
DIFFERENTIAL DIAGNOSIS
- HIV
- Interstitial lung disease
- Lymphoma
- Mycobacterial infection
- Parathyroid disease
[Outline]
PRE-HOSPITAL
Provide supplemental oxygen.
INITIAL STABILIZATION/THERAPY
- Provide supplemental oxygen.
- Monitor for dysrhythmias.
ED TREATMENT/PROCEDURES
- Patients should be observed without therapy, if possible, since disease resolves spontaneously in 50% patients.
- Initiate steroids in patients demonstrating 1 of the following:
- Symptomatic or progressive stage II pulmonary disease
- Stage III pulmonary disease
- Malignant hypercalcemia
- Severe ocular disease
- Neurologic sequelae
- Nasopharyngeal/laryngeal involvement
- Consider topical corticosteroids and cycloplegic agents for anterior uveitis or dermatologic manifestations.
MEDICATION
- Prednisone: 10–80 mg (peds: 0.5–2 mg/kg) PO QD
- Lower doses for hypercalcemic nephropathy and mild to moderate disease
- Higher doses for neurosarcoidosis
[Outline]
DISPOSITION
Admission Criteria
- Hypoxia
- Patients with moderate to severe respiratory symptoms
- Significant cardiac conduction delays
- Severe thrombocytopenia
Discharge Criteria
Follow-up is established.
Issues for Referral
- Cardiology:
- For any conduction disturbances or CHF
- Rheumatology:
- For routine care and follow-up:
- ~q2mo for patients with active disease on steroids, q3–4mo for asymptomatic patients
- Pulmonary:
- For formal pulmonary function testing (to monitor for progression of restrictive lung disease) with spirometry and DLCO
- Ophthalmology:
- Within 48 hr for acute uveitis
FOLLOW-UP RECOMMENDATIONS
- Restrict excess calcium from the diet.
- Monitor for complications related to chronic steroid therapy
[Outline]
- Baughman RP. Pulmonary sarcoidosis. Clin Chest Med. 2004;25:521–530.
- Fauci AS, Braunwald E, Kasper DL, et al. Harrison's principles of internal medicine. 17th ed. New York, NY: McGraw-Hill Professional; 2008.
- Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357:2153–2165.
- King TE. Sarcoidosis. Updated Apr 18, 2011. Available at www.UpToDate.com. Accessed on January 2013.
See Also (Topic, Algorithm, Electronic Media Element)