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Flashcards in Rheumatology/locomotor Deck (29)
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1

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

2

Antibodies associated with: 

A. Limited cutaneous sclerosis 

B. Diffuse cutaneous sclerosis

  • Limited: anti-centromere

 

  • Diffused: anti-scl-70

3

What's CREST syndrome?

CREST → a subtype of limited cutaneous systemic sclerosis

C - calcinosis

R - Raynould's

E - oEsophageal dysmotility

S - sclerodactyly 

T - telangiectasia

4

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

5

X-ray changes in RA 

L - loss/narrowing of joint space

O - juxta-articular osteoporosis

S - subluxation

S - soft tissue swelling

6

X-ray changes in OA

L - loss/narrowing of joint space

O - osteophytes

S - subchondral sclerosis

S - subchondral cysts

7

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

8

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

9

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

10

Compare OA and RA 

11

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

12

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)

13

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

14

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

 

15

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)

16

Name (3) types of medium vessel vasculitis

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

17

Name (2) types of large vessel vasculitis

  • Giant cell arteritis
  • Takayasu’s arteritis

18

What's the most important marker for vasculitis?

Antineutrophil cytoplasmic antibodies (ANCA)

19

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

20

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

21

(4) classic features of Henoch-Schonlein Purpura

  •  purpura 
  • joint pain
  • abdominal pain 
  •  renal involvement 

22

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.

23

Another name for Churg-Strauss syndrome

Eosinophilic granulomatosis with polyangiitis

*small and medium vessel vasculitis

24

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

25

Characteristic features of microscopic polyangiitis 

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

26

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

 

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

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

 

28

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.

29

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.