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Y4 Important to learn > Rheumatology/locomotor > Flashcards

Rheumatology/locomotor Flashcards

1
Q

What body parts are affected in:

A. Limited cutaneous sclerosis

B. Diffuse cutaneous sclerosis

C. Scleroderma

A

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

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2
Q

Antibodies associated with:

A. Limited cutaneous sclerosis

B. Diffuse cutaneous sclerosis

A
  • Limited: anti-centromere
  • Diffused: anti-scl-70
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3
Q

What’s CREST syndrome?

A

CREST → a subtype of limited cutaneous systemic sclerosis

C - calcinosis

R - Raynould’s

E - oEsophageal dysmotility

S - sclerodactyly

T - telangiectasia

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4
Q

Antibodies in systemic sclerosis

A
  • 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
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5
Q

X-ray changes in RA

A

L - loss/narrowing of joint space

O - juxta-articular osteoporosis

S - subluxation

S - soft tissue swelling

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6
Q

X-ray changes in OA

A

L - loss/narrowing of joint space

O - osteophytes

S - subchondral sclerosis

S - subchondral cysts

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7
Q

What problems are associated with Diffuse Cutaneous Systemic Sclerosis?

A

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
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8
Q

What’s meant by scleroderma?

A

Scleroderma →hardening of the skin.

Appearance of:

  • shiny, tight skin without the normal folds in the skin
  • most notable on the hands and face
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9
Q

What’s meant by sclerodactyly?

A

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
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10
Q

Compare OA and RA

A
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11
Q

Management of systemic sclerosis

A

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
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12
Q

Antibodies associated with SLE

A
  • 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)
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13
Q

Possible complications of SLE

A
  • 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 neuritis, transverse myelitis or psychosis
  • Recurrent miscarriage
  • Venous thromboembolism → due to antiphospholipid syndrome occurring secondary to SLE
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14
Q

Treatment of SLE

A

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
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15
Q

Name (4) types of small vessel vasculitis

A
  • Henoch-Schonlein purpura
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (Wegener’s granulomatosis)
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16
Q

Name (3) types of medium vessel vasculitis

A
  • Polyarteritis nodosa
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Kawasaki Disease
17
Q

Name (2) types of large vessel vasculitis

A
  • Giant cell arteritis
  • Takayasu’s arteritis
18
Q

What’s the most important marker for vasculitis?

A

Antineutrophil cytoplasmic antibodies (ANCA)

19
Q

Types of ANCA blood tests associated with vasculitis

A

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
20
Q

Management of vasculitis

A

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
21
Q

(4) classic features of Henoch-Schonlein Purpura

A
  • purpura
  • joint pain
  • abdominal pain
  • renal involvement
22
Q

Management of Henoch Schonlein Purpura

A
  • 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.
23
Q

Another name for Churg-Strauss syndrome

A

Eosinophilic granulomatosis with polyangiitis

*small and medium vessel vasculitis

24
Q

Characteristic features of Churg-Strauss syndrome

A
  • 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
25
Q

Characteristic features of microscopic polyangiitis

A
  • renal failure
  • it can also affect the lungs → shortness of breath and haemoptysis
26
Q

Characteristic features of Wegener’s Granulomatosis

A
  • 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

LRTI:

  • the lungs → a cough, wheeze and haemoptysis.
  • A chest xray may show consolidation and it may be misdiagnosed as pneumonia
  • kidneys → rapidly progressing glomerulonephritis
27
Q

Features of Polyarteritis Nodosa

A

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

28
Q

Features of Kawasaki disease

A

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.

29
Q

Features of Takayasu arteritis

A

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 angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.

Y4 Important to learn (25 decks)

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