Sarcoidosis, Emergency Medicine

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Sarcoidosis, Emergency Medicine

Basics

Description

Unclear, but appears to be an overly robust cell-mediated immune response to unidentified self- or nonself antigen(s) ‚

Chest radiograph (abnormal in 90% sarcoid patients) " ”reason for frequent incidental diagnosis: ‚

• 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%

• Predominance of lung symptoms

Etiology

Diagnosis

Signs and Symptoms

• Constitutional:Fatigue, general weaknessFever
• Skin (25% patients):Rash, lesions
• Cardiac/respiratory (most patients):DyspneaChest painPalpitationsCoughHemoptysis
• Neurologic:Nerve palsy (usually CN VII)SeizureAltered mental status
• Ocular (20% patients):Eye painBlurred vision
• Renal:Flank pain
• Musculoskeletal:Arthralgias

• Fatigue, general weakness
• Fever

• Rash, lesions

• Dyspnea
• Chest pain
• Palpitations
• Cough
• Hemoptysis

• Nerve palsy (usually CN VII)
• Seizure
• Altered mental status

• Eye pain
• Blurred vision

• Flank pain

• Arthralgias

• Constitutional:FeverLethargy
• Skin:Erythema nodosumSubcutaneous nodulesMaculopapulesPlaquesInfiltrative scarsLupus pernio
• EENT:UveitisKeratoconjunctivitisParotid gland enlargement
• Neurologic:Nerve palsy (usually CN VII)
• Respiratory:RalesRarely wheezing
• Cardiac ( ¢ ˆ ¼5% patients):Dysrhythmias, conduction abnormalities, AV blockCHF (due to restrictive cardiomyopathy)Murmurs (due to papillary muscle dysfunction)
• Renal:Nephrolithiasis
• Musculoskeletal:Polyarthralgias
• L ƒ ¶fgren syndrome:Bilateral hilar adenopathyErythema nodosum ‚ ±Polyarthralgias
• Heerfordt " “Waldenstr ƒ ¶m syndrome:FeverUveitisParotid gland enlargement ‚ ±CN VII palsy

• Fever
• Lethargy

• Erythema nodosum
• Subcutaneous nodules
• Maculopapules
• Plaques
• Infiltrative scars
• Lupus pernio

• Uveitis
• Keratoconjunctivitis
• Parotid gland enlargement

• Nerve palsy (usually CN VII)

• Rales
• Rarely wheezing

• Dysrhythmias, conduction abnormalities, AV block
• CHF (due to restrictive cardiomyopathy)
• Murmurs (due to papillary muscle dysfunction)

• Nephrolithiasis

• Polyarthralgias

• Bilateral hilar adenopathy
• Erythema nodosum
• ‚ ±Polyarthralgias

• 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

• 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

• 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

• Biopsy:Bronchoscopy and bronchoalveolar lavageSkin lesions if feasible
• Kveim " “Siltzbach test:Subcutaneous injection of antigen with subsequent spleen biopsyRarely used

• Bronchoscopy and bronchoalveolar lavage
• Skin lesions if feasible

• Subcutaneous injection of antigen with subsequent spleen biopsy
• Rarely used

Differential Diagnosis

• HIV
• Interstitial lung disease
• Lymphoma
• Mycobacterial infection
• Parathyroid disease

Treatment

Pre-Hospital

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 diseaseStage III pulmonary diseaseMalignant hypercalcemiaSevere ocular diseaseNeurologic sequelaeNasopharyngeal/laryngeal involvement
• Consider topical corticosteroids and cycloplegic agents for anterior uveitis or dermatologic manifestations.

• Symptomatic or progressive stage II pulmonary disease
• Stage III pulmonary disease
• Malignant hypercalcemia
• Severe ocular disease
• Neurologic sequelae
• Nasopharyngeal/laryngeal involvement

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

• Hypoxia
• Patients with moderate to severe respiratory symptoms
• Significant cardiac conduction delays
• Severe thrombocytopenia

• 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

• For any conduction disturbances or CHF

• For routine care and follow-up: ¢ ˆ ¼q2mo for patients with active disease on steroids, q3 " “4mo for asymptomatic patients

• ¢ ˆ ¼q2mo for patients with active disease on steroids, q3 " “4mo for asymptomatic patients

• For formal pulmonary function testing (to monitor for progression of restrictive lung disease) with spirometry and DLCO

• 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.

See Also (Topic, Algorithm, Electronic Media Element)

• Dyspnea
• HIV/AIDS
• Hyperparathyroidism
• Tuberculosis

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)