Rheumatology - Polygmyalgia Rheumatica, Raynaud’s phenomenon and Sjogren’s syndrome

Question 1

PR: what is it what and what pts does it primarily affect?

Answer 1

• Clinical syndrome characterized by pain and stiffness of the shoulder, hip girdles, and neck.
• Primarily impacts the elderly, associated with morning stiffness and elevated inflammatory markers

Question 2

PR: who is affected? What is PR associated with and important to watch out for?

Answer 2

Incidence increases with age.

• Average age of onset ~70 years
• Rare in people <50 years of age
• Peak incidence is between ages 70 and 80
• Is associated with Giant Cell Arteritis

Question 3

PR: symptoms from Hx

Answer 3

-Suspect PMR in elderly patients with new
sudden onset of proximal limb pain and
stiffness (neck, shoulders, hips).
- Difficulty rising from chair or combing hair
(proximal muscle involvement)
- Night time pain
- Systemic symptoms in ~25% (fatigue, weight
loss, low-grade fever)

Question 4

PR: symptoms in physical exam

Answer 4

-Decreased range of motion (ROM) of
shoulders, neck, and hips
- Muscle strength is usually normal—may be
limited by pain and/or stiffness.
- Muscle tenderness

Question 5

PR: Dx

Answer 5

• Use Hx and exam and raised inflammatory markers
• Check ESR/CRP
• Consider temporal artery biopsy if symptoms of CGA

Question 6

PR: Rx

Answer 6

• 15 mg daily of prednisolone - usually see response within 5 days
• can give Methotrexate if want to steroid-spare pt

Question 7

What is Raynaud’s phenomenon?

Answer 7

• Condition due to vasospasm of the digits
• Painful
• Characterized by a typical sequence of colour changes in response to a cold stimulus.
• Often also precipitated by stress.

Question 8

What are the causes of Raynaud’s?

Answer 8

Primary: idiopathic - Raynaud’s disease

Secondary (Raynaud’s syndrome)

• Systemic sclerosis, SLE, RA, Sjogren’s, Dermato/polymyositis
• Beta Blockers

Physical

• Heavy vibrating tools
• cervical rib
• ‘Sticky blood’ - cryoglobulinaemia

Question 9

Rx for Raynaud’s

Answer 9

• Keep warm, stop smoking
• 1st line: Calcium-channel blockers
• 2nd line: Phosphodiesterase-5 inhibitors, and prostacyclins
• 3rd line (only some evidence for usefulness of these): ARBs, ACE inhibitors, SSRIs, systemic and topical nitrates.

Question 10

What are the complications of Raynaud’s?

Answer 10

digital ulcers

severe digital ischaemia infection.

Question 11

What is Sjogren’s syndrome?

Answer 11

• Chronic auto-immune inflammatory disorder

- Characterised by finished lacrimal and salivary gland secretion

Question 12

What are the 2 forms/causes of Sjogren’s?

Answer 12

• Primary: not associated with any other diseases
• Secondary: associated with another underlying rheumatological disease (RA and SLE) or auto-immune condition (Coeliac disease, primary biliary cirrhosis, auto-immune thyroid disease)

Question 13

What are common signs/symptoms in Sjogren’s?

Answer 13

• Myalgia
• Arthralgia
• Dry mouth
• Fatigue
• Raynaud’s phenomenon
• Enlarged parotids
• Dry eyes

Question 14

What are important Ix for Sjogren’s?

Answer 14

-Anti-Ro and Anti-La present in 90% of pts
(May also see RF and anti DsDNA but more rare)
-Schirmer’s test: measures tear volume
-Salivary gland biopsy may be needed

Question 15

What happens to pregnant patients with Anti Ro/SS-A Abs?

Answer 15

• At risk of foetal loss
• Complete heart block in Fetus
• Neonatal lupus syndrome in baby

Question 16

What is the Rx for Sjogren’s?

Answer 16

Treat symptoms

• Avoid dry/smoky areas
• Dry eyes: artificial tears
• Dry mouth: artificial saliva, sugar free gum/pastilles
• Skin emollients, vaginal lubricants
• rarely need immunosuppression/steroids