Sjögren syndrome: Difference between revisions
No edit summary |
No edit summary |
||
| Line 1: | Line 1: | ||
==Background== | ==Background== | ||
*Systemic autoimmune | *Systemic autoimmune disorder | ||
*Lymphocytic infiltration of exocrine glands | |||
*Can occur in isolation or with other autoimmune diseases (e.g. [[rheumatoid arthritis]], [[SLE]], etc.) | *Can occur in isolation or with other autoimmune diseases (e.g. [[rheumatoid arthritis]], [[SLE]], etc.) | ||
*Female:male ratio of 9:1 | *Female:male ratio of 9:1 | ||
*10-40 fold increased risk of lymphoma in SS patients. | |||
==Clinical Features== | ==Clinical Features== | ||
*Ocular | *Ocular (xerophthalmia) | ||
** | **Itching, burning sensation | ||
**Gritty, foreign body sensation | **Gritty, foreign body sensation | ||
**[[Dacryoadenitis]] | **[[Dacryoadenitis]] | ||
| Line 21: | Line 23: | ||
*Respiratory | *Respiratory | ||
**Pleuritis | **Pleuritis | ||
**Interstitial lung disease | **Interstitial lung disease | ||
**Obstructive lung disease | **Obstructive lung disease | ||
**[[Pulmonary hypertension]] | **[[Pulmonary hypertension]] | ||
| Line 31: | Line 33: | ||
**[[Cerebral venous thrombosis]] | **[[Cerebral venous thrombosis]] | ||
**Optic nerve injury | **Optic nerve injury | ||
*[[Renal tubular acidosis]] | *Renal | ||
**Interstitial nephritis | |||
**[[Renal tubular acidosis]] | |||
**Hyperchloremic [[metabolic acidosis]] with low bicarb and [[hypokalemia]] | **Hyperchloremic [[metabolic acidosis]] with low bicarb and [[hypokalemia]] | ||
*Cardiac | *Cardiac | ||
**[[Pericarditis]] | **[[Pericarditis]] | ||
**[[Arrhythmia]] due to infiltrative disease | **[[Arrhythmia]] due to infiltrative disease | ||
*Increased risk of [[DVT]]/[[PE]] | *Increased risk of [[DVT]]/[[PE]] | ||
*Gastrointestinal | |||
**Primary biliary cirrhosis | |||
**celiac sprue | |||
| Line 54: | Line 60: | ||
==Evaluation== | ==Evaluation== | ||
===Emergent=== | |||
*Not generally an ED diagnosis | *Not generally an ED diagnosis | ||
*Evaluate for emergent complications | *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== | ==Management== | ||
*Manage emergent complications | *Manage emergent complications | ||
*Topical ocular [[cyclosporine]], artificial tears | *Ocular | ||
* | **Topical ocular [[cyclosporine]], preservative-free artificial tears, lubricating ointments | ||
*Frequent sips of water, chewing gum, or hard candies | *Oral | ||
*Good oral | **Frequent sips of water, chewing gum, or hard candies | ||
*[[Prednisone]] or other | **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) | *AVOID atropine and decongestants (decrease salivary secretions) | ||
Revision as of 21:10, 24 August 2017
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)