25. Sjögren's syndrome and progressive systemic sclerosis

Question 1

What is Sjögren’s syndrome?

Answer 1

• It is an autoimmune disease
  
  • Lymphocytes infiltrate and destroy the lacrimal and salivary glands
  • It’s a multi-organ disease (lungs, skin thyroids, vessels and liver)
• Most commonly seen in women
• Patients have an increased risk of non-hodgkin’s lymphoma

Question 2

What types of Sjögren’s syndrome are there?

Answer 2

Primary:

• Dry eyes and mouth (xerostemia) along with lymphocytic infiltration of the minor saliary glands
• Patients don’t have another rheumatologic disease

Secondary:

• Dry eyes and mouth (xerostemia) along with CT disease (RA, systemic sclerosis, SLE, polymyositis)

Question 3

What are the clinical features of Sjögren’s syndrome?

Answer 3

• Dry eyes, burning, redness, blurred vision
• Xerostemia
• Arthralgias, arthritis, fatigue
• Many extraglandular manifestations (more common in primary disease)
  
  • Chronic arthritis
  • Interstitial nephritis
  • Vasculitis

Question 4

How do we diagnose Sjögren’s syndrome?

Answer 4

• Blood test:
  
  • ANA’s in the blood (95%)
    
    • RF in 75% patients with secondary disease
  • Anti-Ro and Anti-Laantibodies present in 50%
  • Increase ESR
• Schrimer test:
  
  • ​Filter paper is inserted in the eye to measure lacrimal gland output
  • High specificity and sensitivity
• Salivary gland biopsy:
  
  • Lip or parotid
  • Most accurate but not needed for a diagnosis

Question 5

What is the treatment of Sjögren’s syndrome?

Answer 5

• Pilocarpine
• Artificial tears for dry eyes
• NSAIDs, steroids for arthralgia
• If secondary, therapy for CT disease

Question 6

What is systemic sclerosis (scleroderma)?

Answer 6

• Chronic CT disorder that can lead to widespread fibrosis
• More common in women
• Onset: 35-50-years-old
• Raynaud is present in almost all patients

Question 7

What is the pathophysiology of systemic sclerosis?

Answer 7

• Cytokine stimulate fibroblasrs ==> abnormal amount of collagen deposition
  
  • The high quantity of collagen causes problems associated with the disease (composition of collagen is normal)

Question 8

What are the different types of systemic sclerosis?

Answer 8

Diffuse (20%)

Limited (80%)

Question 9

How do we diagnose systemic sclerosis?

Answer 9

• Blood test:
  
  • Almost all patients have elevated ANA antibodies
  • Anti-centromer antibodies is very specific for limited form
  • Antitopoisomerase I antibodies is specific for the diffuse form
• Barrium swallow:
  
  • Used to test esophageal dysmotility complication
• Pulmonary function test:
  
  • To diagnos restrictive lung disease compllication

Question 10

What is the treatment of systemic sclerosis?

Answer 10

• No effective cure
• Symptomatic treatment:
  
  • NSAIDs for muscle pain
  • PPI for esophageal reflux
• Raynaud’s phenomenon:
  
  • Avoid cold
  • Ca²⁺-channel blockers (if severe)
• Treat pulmonary or renal complications (if present)