Sjögren syndrome
(Redirected from Sjögren Syndrome)
Background
- Systemic autoimmune disorder
- Lymphocytic infiltration of exocrine glands
- Can occur in isolation or with other autoimmune diseases (e.g. rheumatoid arthritis, SLE, etc.)
- Female:male ratio of 9:1
- 10-40 fold increased risk of lymphoma in SS patients.
Clinical Features
- Ocular (xerophthalmia)
- Itching, burning sensation
- Gritty, foreign body sensation
- Dacryoadenitis
- Optic neuritis
- Parotid enlargement
- Present in 1/3 of patients
- May be chronic or relapsing
- May lead to suppurative parotitis
- Xerostomia
- Constant thirst
- Dental caries
- Dysphagia
- Pancreatitis
- Respiratory
- Pleuritis
- Interstitial lung disease
- Obstructive lung disease
- Pulmonary hypertension
- Respiratory arrest due to severe hypokalemia
- Small-vessel vasculitis
- Neurologic
- Neuropsychiatric dysfunction
- Transverse myelitis
- Cerebral venous thrombosis
- Optic nerve injury
- Renal
- Interstitial nephritis
- Renal tubular acidosis
- Hyperchloremic metabolic acidosis with low bicarb and hypokalemia
- Cardiac
- Pericarditis
- Arrhythmia due to infiltrative disease
- Increased risk of DVT/PE
- Gastrointestinal
- Primary biliary cirrhosis
- Celiac sprue
Differential Diagnosis
- Sicca complex due to other autoimmune diseases
- Other causes of dry mouth/dry eyes
- Anticholinergic toxicity
- Radiation exposure
- Mumps
- Seasonal allergies
- Cigarette smoke irritation
Evaluation
Emergent
- Not generally an ED diagnosis
- Evaluate for emergent complications
- Thorough physical exam to evaluate for purpura (poor prognosis in primary SS)
Non-Emergent
- Rheumatoid factor is found in 70% of patients
- Anti-SS-A/Ro, and SS-B/La antibodies are part of diagnostic criteria
- Elevated ESR
- Hypergammaglobulinemia
Management
- Manage emergent complications
- Ocular
- Topical ocular cyclosporine, preservative-free artificial tears, lubricating ointments
- Oral
- Frequent sips of water, chewing gum, or hard candies
- Good oral hygiene
- Pylocarpine or Cevimeline for severe xerostomia (parasympathetic and muscarinic agonists)
- Hydroxychloroquine for Sjögren's associated arthritis
- Prednisone or other immunosuppressants (cyclophosphamide or azathioprine) for severe systemic symptoms
- AVOID atropine and decongestants (decrease salivary secretions)