Author:
Jesse B.Cannon
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 (and thus calcium levels) may be increased due to secretion from granulomatous tissue
- Prevalence 10-20/100,000 in the U.S. and Europe
- Affects almost all races and geographic locations
- People of African descent higher predominance
- 2.4% lifetime risk to blacks in the U.S., whites 0.85%
- Symptoms typically begin in patients 10-40 yr of age
Etiology
Unclear, but appears to be an overly robust cell-mediated immune response to unidentified self- or nonself antigen(s). Familial clustering suggests genetic component
Signs and Symptoms
History
- Constitutional:
- Fatigue, weakness
- Weight loss
- Fever
- Skin (25% patients):
- Cardiac/respiratory (90% patients):
- Dyspnea
- Chest pain
- Palpitations
- Cough
- Hemoptysis
- Neurologic:
- Cranial nerve palsy
- Seizure
- Altered mental status
- Ocular (20% patients):
- Eye pain
- Blurred or loss of vision
- Renal:
- Musculoskeletal:
Physical Exam
- Constitutional:
- Skin:
- Erythema nodosum
- Subcutaneous nodules
- Maculopapules
- Plaques
- Infiltrative scars
- Lupus pernio
- EENT:
- Keratoconjunctivitis, uveitis
- 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
Diagnostic Tests & Interpretation
Lab
- Serum ACE elevated in 75% cases
- Leukopenia, eosinophilia possible
- LFTs: Possible mildly elevated alk phos or transaminases
- Serum calcium: Hypercalcemia due to excessive vitamin D
- UA: Hypercalciuria
- Hypergammaglobulinemia
- CSF analysis: Lymphocyte predominance, elevated ACE level
Imaging
- CXR (abnormal in 90% sarcoid patients); reason for frequent incidental diagnosis:
- Stage 1: Bilateral hilar lymphadenopathy
- Stage 2: Bilateral lymphadenopathy and parenchymal lung changes (reticular opacities)
- Stage 3: Parenchymal lung changes, mainly upper, with regressing hilar lymphadenopathy
- Stage 4: Reticular opacities, pulmonary fibrosis; particularly in upper lobes
- Radiotracer scans may identify granulomatous disease but is nonspecific
- CT scans show similar findings as radiographs
Diagnostic Procedures/Surgery
- Biopsy:
- Bronchoscopy and bronchoalveolar lavage
- Skin lesions if feasible
- Kveim-Siltzbach test:
- Subcutaneous injection of antigen, similar to PPD
- Rarely used
Differential Diagnosis
- HIV, CVID
- Other granulomatous disease
- Vasculitis, e.g., Wegener, Churg-Strauss
- Foreign body induced
- Lymphoma
- Fungal infection (e.g., Histoplasmosis)
- Pneumoconiosis
- Mycobacterial infection
- Parathyroid disease
Prehospital
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 per day
- Lower doses for hypercalcemic nephropathy and mild to moderate disease
- Higher doses for neurosarcoidosis
Disposition
Admission Criteria
- Hypoxia
- Patients with moderate to severe respiratory symptoms
- Significant cardiac conduction delays
- Severe thrombocytopenia
Discharge Criteria
Follow-up must be 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
- BaughmanRP. Pulmonary sarcoidosis . Clin Chest Med. 2004;25:521-530.
- FauciAS, BraunwaldE, KasperDL, et al. Harrison's Principles of Internal Medicine. 17th ed.New York: McGraw-Hill Professional; 2008.
- IannuzziMC, RybickiBA, TeirsteinAS. Sarcoidosis . N Engl J Med. 2007;357(21):2153-2165.
- KingTE. Sarcoidosis . www.UpToDate.com. Accessed March 2018.
See Also (Topic, Algorithm, Electronic Media Element)