Sarcoidosis, Emergency Medicine

Basics

Description

Chronic, multisystem disorder characterized by local accumulation of T lymphocytes and mononuclearphagocytes 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) ‚

Diagnosis

Signs and Symptoms

History

Constitutional:
- Fatigue, general weakness
- Fever
Skin (25% patients):
- Rash, lesions
Cardiac/respiratory (most patients):
- Dyspnea
- Chest pain
- Palpitations
- Cough
- Hemoptysis
Neurologic:
- Nerve palsy (usually CN VII)
- Seizure
- Altered mental status
Ocular (20% patients):
- Eye pain
- Blurred vision
Renal:
- Flank pain
Musculoskeletal:
- Arthralgias

Physical Exam

Constitutional:
- Fever
- Lethargy
Skin:
- Erythema nodosum
- Subcutaneous nodules
- Maculopapules
- Plaques
- Infiltrative scars
- Lupus pernio
EENT:
- Uveitis
- Keratoconjunctivitis
- Parotid gland enlargement
Neurologic:
- Nerve palsy (usually CN VII)
Respiratory:
- Rales
- Rarely wheezing
Cardiac ( ¢ ˆ ¼5% patients):
- Dysrhythmias, conduction abnormalities, AV block
- CHF (due to restrictive cardiomyopathy)
- Murmurs (due to papillary muscle dysfunction)
Renal:
- Nephrolithiasis
Musculoskeletal:
- Polyarthralgias
L ƒ ¶fgren syndrome:
- Bilateral hilar adenopathy
- Erythema nodosum
- ‚ ±Polyarthralgias
Heerfordt " “Waldenstr ƒ ¶m syndrome:
- Fever
- Uveitis
- Parotid gland enlargement
- ‚ ±CN VII palsy

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

Treatment

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

Follow-Up

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:
  - ¢ ˆ ¼q2mo 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

Pearls and Pitfalls

Evaluate patients with chest radiographs to determine stage and progression of disease.
Prednisone is treatment of choice for exacerbations of disease.
Monitor for signs of hypercalcemia and related complications.
Be aware of acute neurologic and ocular sequelae.

Additional Reading

Baughman ‚ RP. Pulmonary sarcoidosis. Clin Chest Med. 2004;25:521 " “530.
Fauci ‚ AS, Braunwald ‚ E, Kasper ‚ DL, et al. Harrisons 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.

Codes

ICD9

135 Sarcoidosis
517.8 Lung involvement in other diseases classified elsewhere

ICD10

D86.0 Sarcoidosis of lung
D86.3 Sarcoidosis of skin
D86.9 Sarcoidosis, unspecified
D86.85 Sarcoid myocarditis
D86.1 Sarcoidosis of lymph nodes
D86.2 Sarcoidosis of lung with sarcoidosis of lymph nodes
D86.81 Sarcoid meningitis
D86.82 Multiple cranial nerve palsies in sarcoidosis
D86.83 Sarcoid iridocyclitis
D86.84 Sarcoid pyelonephritis
D86.86 Sarcoid arthropathy
D86.87 Sarcoid myositis
D86.89 Sarcoidosis of other sites
D86.8 Sarcoidosis of other sites
D86 Sarcoidosis

SNOMED

31541009 Sarcoidosis (disorder)
24369008 Pulmonary sarcoidosis (disorder)
55941000 cutaneous sarcoidosis (disorder)
75403004 Cardiac sarcoidosis
193251003 Sarcoid myopathy
230193008 Neurosarcoidosis
234526006 Ocular sarcoidosis (disorder)
64757003 lymph node sarcoidosis (disorder)

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