Sjogren's syndrome

Question 1

What are the clinical features of sjogren’s syndrome?

Answer 1

1°: a/w HLA-B8/DR3

2° to RA, and other autoimmune diseases – many Rheumatological Conditions can predispose Sjogrens!

Question 2

What are the causes of sjogren’s syndrome?

Answer 2

• Common symptoms
• Fatigue and sicca symptoms (refers spef to dry eyes, mouth, skin, vagina)
• +/- Diffuse Bilateral Parotidomegaly -> to check for Facial N Invovlement!

Less common systemic symptoms

• Arthralgia or non-progressive polyarthritis (like SLE; much less common)
• Raynaud’s phenomenon
• Dysphagia, abnormal oesophageal dysmotility (less common)
• Other autoimmune disease: thyroid, myasthenia gravis, PBC, autoimmune hepatitis, pancreatitis
• Renal tubular defects (uncommon): nephrogenic DI, RTA
• Pulmonary diffusion defects, fibrosis
• Polyneuropathy, fits, depression
• Vasculitis
• ↑ incidence of non-Hodgkin’s B-cell lymphoma -> hence impt to f/u despite mild S&S; Assess for B Symptoms

Question 3

What are the investigations for sjogren’s disease?

Answer 3

Schirmer tear test (sounds like 什么)

• Filter paper placed on inside of lower eyelid
• Wetting of <10mm in 5 min: defective tear production

Reduced salivary pooling

Rose Bengal staining of eyes
- Punctate or filamentary keratitis

Lab abnormalities

• ↑ IgG (monitor titres): note isolated IgG not dx of Sjogren’s, as pts may have high titres w/o ever being symptomatic
• Anti-Ro (60-90%), anti-La, RF +ve, ANA (80%), anti-mitochondrial (10%)
• Anti-Ro (SSA) may cross placenta and cause congenital heart block

Question 4

What is the management of sjogren’s disease?

Answer 4

• Symptomatic: artificial tears and saliva replacement solutions
• Hydroxychloroquine: may help fatigue and arthralgia
• Corticosteroids (rarely needed): for persistent salivary gland swelling or neuropathy