Cortex rheumatology - Inflammatory arthropathies 5

Question 1

What investigations are done to help diagnose polymyositis and dermatomyositis ?

Answer 1

• Inflammatory markers are often raised.
• Serum creatine kinase (CK) level is usually raised, often more than 10 times the normal level.
• Anti-Jo-1 and anti-SRP (these are the antibodies more specific to myositis)
• MRI may be useful to localize the extent of muscle involvement.
• Electromyographic (EMG) (abnormal in 90% of patients with it)
• Muscle biopsy is crucial in helping to diagnose polymyositis and in excluding other rare muscle diseases (believe this sounds the gold standard)

Question 2

What is the treatment of polymyositis ?

Answer 2

prednisolone (initially around 40mg) combined with immunosuppressive drugs such as methotrexate or azathioprine.

Question 3

What are the features shown and what condition are they associated with ?

Answer 3

1. Heliotrope rash
2. Gottons papules
3. V-shaped rash over chest

Dermatomyositis

Question 4

What is fibromyalgia ?

Answer 4

An unexplained condition causing widespread muscle pain and fatigue

Question 5

Describe the presentation of fibromyalgia

Answer 5

Fibromyalgia is a syndrome that consists of the following signs and symptoms:

• Persistent (≥ 3 months) widespread pain (pain/tenderness on both sides of the body, above and below the waist, and includes the axial spine
• Fatigue; disrupted and unrefreshing sleep
• Cognitive difficulties
• Multiple other unexplained symptoms, anxiety and/or depression, and functional impairment of activities of daily living (ADLs)

Question 6

How is fibromyalgia diagnosed ?

Answer 6

Diagnosis made on a clincial basis - important to rule out any other possible conditions

Question 7

What is the management of fibromyalgia ?

Answer 7

Mainstay of therapy to for the patient to learn self-management techniques and understand the condition. It is important to validate the patient’s illness through listening and acknowledgment that the patient is indeed experiencing pain

Question 8

Define what is meant by vasculitis and what it can result in

Answer 8

• Defined as inflammation of blood vessels
• May result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia.

Question 9

What are the different categories of vasculitis ? (think in terms of size)

Answer 9

• Large vessel vasculitis
• Small to medium vessel vasculitis

Question 10

What are the two main large vessel vasculitis ?

Answer 10

GCA and Takayasu arteritis

Question 11

What is the main difference between GCA and takayasu vasculitis ?

Answer 11

• GCA rarley occurs before 50
• Takayasu rarley occurs after 50

Question 12

Describe in general terms thinking about the pathology what is meant by large vessel vasculitis

Answer 12

Chronic granulomatous inflammation predominantly of the aorta and its major branches.

Question 13

Describe the early and late symptoms associated with large vessel vasculitis (this is a bit more general than the specific ones earlier about GCA but still similar)

Answer 13

Early features:

• Low-grade fever, malaise
• Night sweats
• Weight loss
• Arthralgia
• Fatigue.

Following this patients can experience claudicant symptoms in both the upper and lower limbs.

Question 14

What are the investigations appropriate to takayasu arteritis ?

Answer 14

CRP, ESR/PV all raised

CT angiogram or MR angiogram to detect for aneurysms or vascular stenosis

Question 15

What is the treatment of GCA and takayasu’s arteritis ?

Answer 15

Main stay is Prednisolone

If needed immunosuppressants may be added:

• 1st line - methotrexate
• 2nd line - azathioprine

Question 16

How can small to medium vessel vasculitis be further classified ?

Answer 16

Into ANCA positive or negative conditions

Question 17

What are the 4 main types of ANCA positive vasculitis ?

Answer 17

• Granulomatosis with polyangiitis (GPA)
• Microscopic polyangiitis (MPA)
• EGPA (eosinophilic granulomatosis with polyangiitis) - also known as churg strauss syndrome
• Renal limited vasculitis (RLV)

Think ‘GERM’

Question 18

What are the general features associated with ANCA positive vascultiis ?

Answer 18

• Fever and weight loss
• A raised, non blanching purpuric rash
• Arthralgia/arthritis
• Mononeuritis multiplex - type of nephropathy due to nerve damage
• Glomerulonephritis
• Lung opacities on x-ray

Question 19

What is GPA characterised by ?

Answer 19

ENT symptoms common:

Include nose bleeds, deafness, recurrent sinusitis and nasal crusting (over time there can be collapse of the nose). Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray are frequently seen.

associated with cANCA and PR3 (proteinase 3) antibodies

Question 20

What is EGPA characterised by ?

Answer 20

Characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count.

Question 21

What condition is strongly linked with microscopic polyangiitis ?

Answer 21

Glomerulonephritis

Question 22

What antibodies is positive in MPA and EGPA (more so in MPA)

Answer 22

MPO-ANCA

Question 23

What investigations are useful in helping diagnose ANCA positive vasculitis ?

Answer 23

• ESR, PV and CRP and raised (remember inflammatory condition)
• Anaemia of chronic disease is common.
• U+E looking for renal involvement
• Anti-neutrophil cytoplasmic antibody (ANCA)
• Urinalysis (looking for renal vasculitis)
• CXR
• Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis

Question 24

What is the most common treatment for ANCA associated vasculitis ?

Answer 24

Due to aggressive nature usually require:

IV steroids and cyclophosphamide

Question 25

What is Henoch-Schonlein purpura?

Answer 25

• Small vessel vasculitis
• It is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis

Question 26

What are the characteristic features of Henoch-Schonlein purpura?

Answer 26

It commonly affects children. Commonly a history of an upper respiratory tract infection predates the symptoms by a few weeks.

Common symptoms:

• Purpuric rash over the buttocks and lower limbs
• Abdominal pain
• Vomiting and joint pain.

Question 27

What is the treatment of Henoch-Schonlein purpura?

Answer 27

Usually self-limiting