MSK and Rheum - Polymyalgia Rheumatica, Temporal Arteritis, Polyarteritis Nodosa

Question 1

Polymyalgia Rheumatica - what is it?

Answer 1

Condition characterised by muscle stiffness and raised inflammatory markers

Question 2

Polymyalgia Rheumatica - who does it affect and how quickly?

Answer 2

Commonly >60yo

Rapid onset <1 month

Question 3

Polymyalgia Rheumatica - what are the clinical features?

Answer 3

MORNING STIFFNESS in proximal limb muscles

Weakness is NOT considered a symptom

Low-grade fever, night sweats

Polyarthralgia

Question 4

Polymyalgia Rheumatica - what are the investigations and the associated blood product levels?

Answer 4

Rasided inflamm markers = ESR >40mm/hr

Creatine Kinase (CK) = NORMAL, way to differentiate from other disorders

Question 5

Polymyalgia Rheumatica - management?

Answer 5

PREDNISOLONE 15mg OD

Patients should respond dramatically to steroids - so if they don’t consider diff diagnosis

Question 6

Temporal Arteritis - what is it?

Answer 6

It is a large vessel vasculitis

Overlaps with polymyalgia rheumatica

Question 7

Temporal Arteritis - what are the overlapping features with PMR?

Answer 7

Older people >60yo

Rapid onset <1 month

Clinical features - 50% have features of PMR:
1. Morning stiffness, but not weakness

Question 8

Temporal Arteritis - what are the clinical features?

Answer 8

1. Morning stiffness in proximal limb muscles, but NOT WEAKNESS
2. VISUAL DISTURBANCES - double vision, amaurosis fugax, blurring
3. Headache
4. Jaw claudication
5. Tender, palpable temporal artery

Question 9

Temporal Arteritis - what are the investigations and what would you see?

Answer 9

Bloods:
1. Inflamm markers RAISED = ESR >50mm/hr

2. Temporal artery biopsy - SKIP LESIONS PRESENT
3. VISION TESTING - results changes initial management step
4. CK = NORMAL, like in PMR
5. EMG = normal

Question 10

Temporal Arteritis - management?

Answer 10

Urgent glucocorticoids given before biopsy, as soon as TA suspected:

1. If NO vision disturbances = high-dose prednisolone
2. If there IS visual disturbances = IV methylprednisolone given first, then high-dose prednisolone
3. Same day ophthalmology review
   - Consider giving bisphosphonates as patient on long-term course of steroids (risk of osteoporosis)

Question 11

Polyarteritis Nodosa (PAN) - what is it?

Answer 11

It is a vasculitis affecting medium sized arteries with necrotizing inflammation leading to aneurysm formation

Affects many organs

Associated with HepB

Question 12

PAN - what is the characteristic disease it is associated with?

Answer 12

HepB

Question 13

PAN - when does it present?

Answer 13

Presents more in men, aged 40-60yo

Question 14

PAN - what are the clinical features?

Answer 14

Kidney – renal failure, HAEMATURIA

Coronary – ischemic heart disease, acute myocardial infarction

GI – abdominal pain, nausea, melaena, WEIGHT LOSS

Musculoskeletal – arthritis, myalgia, arthralgia

Skin - LIVEDO RETICULARIS

CNS – eye and skin complaints

Pulmonary - pulmonary vessels are NOT affected in PAN (‘P’ulmonary vessels ‘A’re ‘N’OT affected, so PAN)

Question 15

PAN - what are the investigations?

Answer 15

Hepatitis B serology positive in 30% of patients

Diagnosis challenging as features not distinguishable from other diseases

Diagnosis based on symptoms described, physical examination, lab tests (to exclude other causes)

Possibly biopsy of the affected area

Question 16

PAN - management?

Answer 16

1. Corticosteroid therapy
2. If relapse whilst on corticosteroids, add cyclophosphamide
3. Maintenance therapy - azathioprine