Cortex rheumatology - Inflammatory arthropathies 5 Flashcards
What investigations are done to help diagnose polymyositis and dermatomyositis ?
- 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)
What is the treatment of polymyositis ?
prednisolone (initially around 40mg) combined with immunosuppressive drugs such as methotrexate or azathioprine.
What are the features shown and what condition are they associated with ?
- Heliotrope rash
- Gottons papules
- V-shaped rash over chest
Dermatomyositis
What is fibromyalgia ?
An unexplained condition causing widespread muscle pain and fatigue
Describe the presentation of fibromyalgia
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)
How is fibromyalgia diagnosed ?
Diagnosis made on a clincial basis - important to rule out any other possible conditions
What is the management of fibromyalgia ?
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
Define what is meant by vasculitis and what it can result in
- Defined as inflammation of blood vessels
- May result in vessel wall thickening, stenosis, and occlusion with subsequent ischemia.
What are the different categories of vasculitis ? (think in terms of size)
- Large vessel vasculitis
- Small to medium vessel vasculitis
What are the two main large vessel vasculitis ?
GCA and Takayasu arteritis
What is the main difference between GCA and takayasu vasculitis ?
- GCA rarley occurs before 50
- Takayasu rarley occurs after 50
Describe in general terms thinking about the pathology what is meant by large vessel vasculitis
Chronic granulomatous inflammation predominantly of the aorta and its major branches.
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)
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.
What are the investigations appropriate to takayasu arteritis ?
CRP, ESR/PV all raised
CT angiogram or MR angiogram to detect for aneurysms or vascular stenosis
What is the treatment of GCA and takayasu’s arteritis ?
Main stay is Prednisolone
If needed immunosuppressants may be added:
- 1st line - methotrexate
- 2nd line - azathioprine
How can small to medium vessel vasculitis be further classified ?
Into ANCA positive or negative conditions
What are the 4 main types of ANCA positive vasculitis ?
- Granulomatosis with polyangiitis (GPA)
- Microscopic polyangiitis (MPA)
- EGPA (eosinophilic granulomatosis with polyangiitis) - also known as churg strauss syndrome
- Renal limited vasculitis (RLV)
Think ‘GERM’
What are the general features associated with ANCA positive vascultiis ?
- 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
What is GPA characterised by ?
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
What is EGPA characterised by ?
Characterised by late onset asthma, rhinitis and a raised peripheral blood eosinophil count.
What condition is strongly linked with microscopic polyangiitis ?
Glomerulonephritis
What antibodies is positive in MPA and EGPA (more so in MPA)
MPO-ANCA
What investigations are useful in helping diagnose ANCA positive vasculitis ?
- 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
What is the most common treatment for ANCA associated vasculitis ?
Due to aggressive nature usually require:
IV steroids and cyclophosphamide
What is Henoch-Schonlein purpura?
- Small vessel vasculitis
- It is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalised vasculitis
What are the characteristic features of Henoch-Schonlein purpura?
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.
What is the treatment of Henoch-Schonlein purpura?
Usually self-limiting
