Rosen & Barkin's 5-Minute Emergency Medicine Consult (630 page)

• Patients need to maintain their respiratory drive to reverse acidemia, respiratory acidosis:
  
  • Do not intubate prematurely.
    
  • It is extremely difficult to achieve and maintain mechanical hyperventilation in these patients.
    
• Salicylate poisoning may result from topical exposure to salicylate-containing lotions or creams, rectal suppositories, oral antidiarrheal preparations.
  
• Salicylate levels may trend downward only to begin increasing again due to absorption of product from the intestine or from a salicylate bezoar in the gut.
  

• Kent K, Ganetsky M, Cohen J, et al. Non-fatal ventricular dysrhythmias associated with severe salicylate toxicity.
  Clin Toxicol (Phila)
  . 2008;46:297–299.
  
• Stolbach AI, Hoffman RS, Nelson LS. Mechanical ventilation was associated with acidemia in a case series of salicylate-poisoned patients.
  Acad Emerg Med
  . 2008;15:866–869.
  
• West PL, Horowitz BZ. Delayed recrudescence to toxic salicylate concentrations after salsalate overdose.
  J Med Toxicol
  . 2010;6:150–154.
  

CODES

• 276.2 Acidosis
  
• 276.3 Alkalosis
  
• 965.1 Poisoning by salicylates
  

BASICS

• 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%
  

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

DIAGNOSIS

• 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
    

• 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 (∼5% patients):
  
  • Dysrhythmias, conduction abnormalities, AV block
    
  • CHF (due to restrictive cardiomyopathy)
    
  • Murmurs (due to papillary muscle dysfunction)
    
• Renal:
  
  • Nephrolithiasis
    
• Musculoskeletal:
  
  • Polyarthralgias
    
• Löfgren syndrome:
  
  • Bilateral hilar adenopathy
    
  • Erythema nodosum
    
  • ±Polyarthralgias
    
• Heerfordt–Waldenström 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.
  

• Physical exam with emphasis on lung, skin, eye, heart, and musculoskeletal
  
• Pulse oximetry/ABG
  
• ECG (dysrhythmias, conduction delays)
  
• Slit-lamp eye exam
  

• 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
  

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
  

• Biopsy:
  
  • Bronchoscopy and bronchoalveolar lavage
    
  • Skin lesions if feasible
    
• Kveim–Siltzbach test:
  
  • Subcutaneous injection of antigen with subsequent spleen biopsy
    
  • Rarely used
    

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

TREATMENT

Provide supplemental oxygen.

• Provide supplemental oxygen.
  
• Monitor for dysrhythmias.
  

• 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.
  

• 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

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

Follow-up is established.

• 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