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Flashcards in Rheumatology/locomotor Deck (29)
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What body parts are affected in: 

A. Limited cutaneous sclerosis 

B. Diffuse cutaneous sclerosis 

C. Scleroderma

A. Limited:  Face + distal limbs + CREST syndrome


B.Diffuse: trunk + proximal limbs + Interstitial lung disease/ pulmonary arterial hypertension


C. Scleroderma → only the skin is affected


Antibodies associated with: 

A. Limited cutaneous sclerosis 

B. Diffuse cutaneous sclerosis

  • Limited: anti-centromere


  • Diffused: anti-scl-70


What's CREST syndrome?

CREST → a subtype of limited cutaneous systemic sclerosis

C - calcinosis

R - Raynould's

E - oEsophageal dysmotility

S - sclerodactyly 

T - telangiectasia


Antibodies in systemic sclerosis

  • ANA positive in 90%
  • RF positive in 30%
  • anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
  • anti-centromere antibodies associated with limited cutaneous systemic sclerosis


X-ray changes in RA 

L - loss/narrowing of joint space

O - juxta-articular osteoporosis

S - subluxation

S - soft tissue swelling


X-ray changes in OA

L - loss/narrowing of joint space

O - osteophytes

S - subchondral sclerosis

S - subchondral cysts


What problems are associated with Diffuse Cutaneous Systemic Sclerosis ?

Features of CREST syndrome plus many internal organs causing:

  • Cardiovascular problems, particularly hypertension and coronary artery disease


  • Lung problems, particularly pulmonary hypertension and pulmonary fibrosis


  • Kidney problems, particularly glomerulonephritis and scleroderma renal crisis


What's meant by scleroderma?

Scleroderma →hardening of the skin.

Appearance of:

- shiny, tight skin without the normal folds in the skin

- most notable on the hands and face


What's meant by sclerodactyly?

Sclerodactyly → the skin changes in the hands

  • skin tightens around joints → it restricts the range of motion in the joint and reduces the function of the joints
  •  skin hardens and tightens further → the fat pads on the fingers are lost
  • The skin can break and ulcerate


Compare OA and RA 


Management of systemic sclerosis 

Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis


Non-medical management involves:

  • Avoid smoking
  • Gentle skin stretching to maintain the range of motion
  • Regular emollients
  • Avoiding cold triggers for Raynaud’s
  • Physiotherapy to maintain healthy joints
  • Occupational therapy for adaptations to daily living to cope with limitations


Medical management focuses on treating symptoms and complications:

  • Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
  • Anti-acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
  • Analgesia for joint pain
  • Antibiotics for skin infections
  • Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
  • Treatment of pulmonary artery hypertension
  • Supportive management of pulmonary fibrosis


Antibodies associated with SLE

  • Anti-nuclear antibodies (ANA) positive 85% of SLE cases (but not specific)


  • Anti-double stranded DNA (anti-dsDNA) is specific to SLE; positive in 70% of patients with SLE 


  • Anti-Smith (highly specific to SLE)


  • Anti-centromere antibodies (most associated with limited cutaneous systemic sclerosis)


  • Anti-Ro and Anti-La (most associated with Sjogren’s syndrome)


  • Anti-Scl-70 (most associated with systemic sclerosis)


  • Anti-Jo-1 (most associated with dermatomyositis)


Possible complications of SLE 


  • Cardiovascular disease → chronic inflammation in the blood vessels → hypertension and coronary artery diseas


  • Infection (part of the disease process and secondary to immunosuppressants


  • Anaemia of chronic disease → affects the bone marrow causing a chronic normocytic anaemia


  • leukopenia, neutropenia, thrombocytopenia


  • Pericarditis 
  • Pleuritis 
  • Interstitial lung disease → caused by inflammation in the lung tissue → pulmonary fibrosis
  • Lupus nephritis → inflammation in the kidney →  end-stage renal failure


  • Neuropsychiatric SLE → inflammation in the central nervous system (optic neuritistransverse myelitis or psychosis


  • Recurrent miscarriage 


  • Venous thromboembolism → due to antiphospholipid syndrome occurring secondary to SLE


Treatment of SLE

First-line treatments are:

  • NSAIDs
  • Steroids (prednisolone)
  • Hydroxychloroquine (first line for mild SLE)
  • Suncream and sun avoidance for the photosensitive the malar rash

Other commonly used immunosuppressants in resistant or more severe lupus:

  • Methotrexate
  • Mycophenolate mofetil
  • Azathioprine
  • Tacrolimus
  • Leflunomide
  • Ciclosporin

Biological therapies (considered for patients with severe disease or where patients have not responded to other treatments): 

  • Rituximab is a monoclonal antibody that targets the CD20 protein on the surface of B cells
  • Belimumab is a monoclonal antibody that targets B-cell activating factor



Name (4) types of small vessel vasculitis

  • Henoch-Schonlein purpura
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (Wegener’s granulomatosis)


Name (3) types of medium vessel vasculitis

  • Polyarteritis nodosa
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Kawasaki Disease


Name (2) types of large vessel vasculitis

  • Giant cell arteritis
  • Takayasu’s arteritis


What's the most important marker for vasculitis?

Antineutrophil cytoplasmic antibodies (ANCA)


Types of ANCA blood tests associated with vasculitis 

There are two type of ANCA blood tests: p-ANCA and c-ANCA

  • p-ANCA (PR3 antibodies): Microscopic polyangiitis and Churg-Strauss syndrome
  • c-ANCA (MPO antibodies): Wegener’s granulomatosis


Management of vasculitis 

Rheumatology referral - to diagnose + treat

Combination of steroids and immunosuppressants.

Steroids can be administered to target the affected area:

  • Oral (i.e. prednisolone)
  • Intravenous (i.e. hydrocortisone)
  • Nasal sprays for nasal symptoms
  • Inhaled for lung involves (e.g. Churg-Strauss syndrome)

Immunosuppressants that are used include:

  • Cyclophosphamide
  • Methotrexate
  • Azathioprine
  • Rituximab and other monoclonal antibodies


(4) classic features of Henoch-Schonlein Purpura

  •  purpura 
  • joint pain
  • abdominal pain 
  •  renal involvement 


Management of Henoch Schonlein Purpura

  •  typically supportive → simple analgesia, rest and proper hydration

- abdominal pain usually settles within a few days

- patients without kidney involvement can expect to fully recover within 4-6 weeks

- a third of patients have a recurrence of the disease within 6 months

- 1% of patients will go on to develop end stage renal failure.


Another name for Churg-Strauss syndrome

Eosinophilic granulomatosis with polyangiitis

*small and medium vessel vasculitis


Characteristic features of Churg-Strauss syndrome

  • lung and skin problems (but can affect other organs such as kidneys)


  • often presents with severe asthma in late teenage years or adulthood


  • a characteristic finding is elevated eosinophil levels on the full blood count


Characteristic features of microscopic polyangiitis 

  • renal failure
  • it can also affect the lungs → shortness of breath and haemoptysis


Characteristic features of Wegener's Granulomatosis 

  •  affects the respiratory tract and kidneys

URTI affects:

  •  nose → nose bleeds (epistaxis) and crusty nasal secretions
  • ears → hearing loss
  • sinuses→ sinusitis

A classic sign in exams is the saddle-shaped nose due to a perforated nasal septum



  •  the lungs → a cough, wheeze and haemoptysis.
  • A chest xray may show consolidation and it may be misdiagnosed as pneumonia


  • kidneys → rapidly progressing glomerulonephritis


Features of Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is a medium vessel vasculitis

  • associated with hepatitis B but can also occur without a clear cause or with hepatitis C and HIV.


  • affected vessels in: skin, gastrointestinal tract, kidneys and heart → renal impairment, strokes and myocardial infarction


(!) It is associated with a rash called livedo reticularis → mottled, purplish, lace like rash



Features of Kawasaki disease

Kawasaki disease is a medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.

Clinical features are:

  • Persistent high fever > 5 days
  • Erythematous rash
  • Bilateral conjunctivitis
  • Erythema and desquamation (skin peeling) of palms and soles
  • Strawberry tongue” (red tongue with prominent papillae)

A key complication is coronary artery aneurysms.

Treatment is with aspirin and IV immunoglobulins.


Features of Takayasu arteritis

Takayasu’s arteritis is a form of large vessel vasculitis.

It mainly affects:

- the aorta and it’s branches

- pulmonary arteries

These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. This leads to it’s other name of “pulseless disease”.

Presentation: Japanese female below 40 y old; non-specific systemic symptoms (fever, malaise and muscle aches) or with more specific symptoms of arm claudication or syncope.

Diagnosis:  CT or MRI angiographyDoppler ultrasound of the carotids can be useful in detecting carotid disease.