Sjögren syndrome
Background
- Systemic autoimmune disease
- Can occur in isolation or with other autoimmune diseases (e.g. rheumatoid arthritis, SLE, etc.)
- Female:male ratio of 9:1
Clinical Features
- Ocular
- 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 tubular acidosis
- Hyperchloremic metabolic acidosis with low bicarb and hypokalemia
- Chronic interstitial nephritis
- Cardiac
- Pericarditis
- Arrhythmia due to infiltrative disease
- Increased risk of DVT/PE
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
- Not generally an ED diagnosis
- Rheumatoid factor is found in 70% of patients
- May also have anti-SS-A/SS-B antibodies
- Evaluate for emergent complications
Management
- Manage emergent complications
- Topical ocular cyclosporine, artificial tears
- Pylocarpine and cevimeline for severe xerostomia
- Frequent sips of water, chewing gum, or hard candies
- Good oral hygeine
- Prednisone or other immunosupressives for severe systemic symptoms
- AVOID atropine and decongestants (decrease salivary secretions)