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MRCP PACES revision > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Autoantibodies in SLE

A
  1. ANA
  2. dsDNA (high spec, varies with severity)
  3. Nucleosome (high spec)
  4. Ro (Sjogren’s)
  5. La (Sjogren’s)
  6. Sm (high spec)
  7. U1RNP
  8. rNP
  9. Histone (Drug induced)
  10. Antiphospholipid
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2
Q

What is the epidemiology of SLE?

A

Female
Black > Asian > Caucasian
Rare 4/100,000/yr

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3
Q

Body systems affected by SLE

A 
Constitutional symptoms
Dermatological
Musculoskeletal
Cardiovascular
Respiratory
Neurological
Renal
Haematological
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4
Q

General/Constitutional symptoms in SLE

A 
Fatigue
Anorexia
Weight loss
Low grade fever
Lymphadenopathy
Serositis (pleural/pericardial)
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5
Q

Musculoskeletal symptoms of SLE

A
  1. Arthralgia (90%)
    - Synovitis
    - Effusions
    - Morning stiffness
    - Jaccoud’s arthropathy
  2. Myalgia
  3. Tenosynovitis
  4. Osteoporosis
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6
Q

Clinical course of SLE

A
  • Unpredictable
  • Acute flares
  • Periods of apparent remission
  • Long periods of subclinical inflammatory activity
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7
Q

Dermatological manifestations of lupus

A 
Malar Rash
Oral ulcers
Raynaud's phenomenon
Discoid lupus
Non and Scarring alopecia
Purpura/vasculitic lesions
Livedo reticularis (APL abs)
Bullous lupus
Toxic epidermal SLE
Maculopapular lupus rash
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8
Q

Cardiovascular manifestations of lupus

A 
Pericarditis
Myocarditis
Heart block
Pericardial effusion
Libman Sacks Endocarditis
Increase IHD risk
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9
Q

Renal manifestations of lupus

A

Glomerulonephritis (often severe)

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10
Q

Neurological manifestations of lupus

A 
Headache (migraine/BIH/HTN)
Acute confusional state/Psychosis
Transverse myelitis
Mononeuritis multiplex
Peripheral/cranial neuropathy
Seizures
Anxiety/depression
Stroke
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11
Q

Respiratory manifestations of lupus

A 
Pleural effusions
Pleurisy
Shrinking lung syndrome
Interstitial fibrosis/pneumonitis
Pulmonary HTN
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12
Q

Haematological manifestations of lupus

A 
Anaemia
-Haemolytic
-Of chronic disease
Lymphopenia
Thrombocytopenia
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13
Q

Autoimmune conditions associated with SLE

A 
Sjogren's
Antiphospholipid syndrome
Thyroid disease
RA
MG
DM
Pernicious anaemia
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14
Q

Gastrointestinal manifestations of lupus

A 
Abdo pain
Nausea
Intestinal vasculitis
Hepatitis
Pancreatitis
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15
Q

Drugs causing lupus

A 
Hydralazine
Minocycline
TNF alpha biologics (infliximab)
Lithium
Anticonvulsants
Isoniazid
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16
Q

What is systemic sclerosis

A

Spectrum of disorders with

  1. Skin Sclerosis
  2. Raynaud’s

Spectrum:

  1. Localised cutaneous scleroderma
  2. Systemic Sclerosis
    - -LimIted cutaneous (formerly CREST)
    - -Diffuse cuteanous
  3. Raynaud’s Phenomenon
    - -Autoimmune
    - -Primary
17
Q

Autoantibodies/Complement/Immunoglobulins in systemic sclerosis

A

Diffuse cutaneous

  • -Scl-70 (anti-topoisomerase)
  • -Anti RNA polymerase III (RNAP)

Limited cutaneous:
–Anti-centromere

Hypergammaglobulinaemia
Reduced complement

18
Q

What is the pathogenesis of systemic sclerosis

A

Vascular abnormalities - endothelial cell injury and increased ECM in vessel wall

Connective tissue fibrosis

  • Fibrogenic fibroblasts
  • increased ECM - disrupts tissue architecture
19
Q

What are the clinical features of localised cutaneous scleroderma

A

“Morphoea”

  • -Localised: 1 or more skin lesions - often trunk
  • -Generalised: without raynaud/viscera involvement
  • -En coup de sabre - midline lesion in childhood defect
  • -Linear - dermatomal distribution, childhood
20
Q

What are the clinical features of limited cutaneous systemic sclerosis?

A 
C alcinosis
R aynauds (usually precedes other symptoms by years)
E sophageal dysmotility
S cleroderma: face/neck/hands
T elangiectasia

Other viscera involvement:
Lung
Renal
Heart

21
Q

What are the clinical features of diffuse cutaneous systemic sclerosis

A
  • Presents abruptly with skin changes over 1-3y
  • Pruritus
  • Constitutional (lethargy, weight loss)
  • Scleroderma: trunk, acral, pigment change
  • Raynaud’s - develops later

Visceral disease:

  • Oesophagus
  • Lungs (fibrosis, PAH)
  • Renal crisis (HTN, headache)
  • Heart
22
Q

What is the classification of Raynaud’s phenomenon?

A
  1. Autoimmune
    - Antibodies (RA, SLE, Sclerosis, myositis)
    - Abnormal nailfold
  2. Primary
    - Normal Antibodies and capillaroscopy
23
Q

What is the differential of Raynaud’s?

A
  • Raynaud
  • Haematological
  • –Cryoglobulinaemia
  • –Cold agglutinin disease
  • –Hyperviscosity syndrome
  • Macrovascular disease
  • Vasculitis
24
Q

How are the viscera effected in sclerosis?

A

Respiratory

  • Fibrosis (NSIP)
  • PAH
  • Pleural disease (effusions/pleurisy)

GI

  • Mouth - tight skin, sicca
  • Oesophagus - dysmotility, spasm, strictures
  • Stomach - gastroparesis
  • Bowel - hypo motility, stasis, volvulus
  • Anus - incontinence

Cardiac

  • Fibrosis - arrhythmias, heart block
  • Pericarditis/myocarditis

Renal

  • Glomerulonephritis
  • Chronic nephropathy
  • Renal crisis
25
Q

How is systemic sclerosis managed?

A 
MDT approach - refer to appropriate specialties
Immunomodulation:
-Steroids (esp. pulmonary fibrosis)
-Cyclophosphamide
-MMF
26
Q

How is Raynaud’s managed?

A

Non-pharmacological:

  • Hand warmers
  • Warm clothing
  • Primrose oil

Vasodilators

  • Calcium channel blockers
  • Losartan
  • Topical GTN

Severe (ulcers/necrosis):
-Iloprost

27
Q

How is a scleroderma renal crisis managed?

A
  1. ACEi (70 –> 10% mortality)

2. RRT

28
Q

What are the symptoms of rheumatoid arthritis?

A
  • Weeks to months onset
  • Stiffness - worse in morning/inactivity
  • Pain
  • Swelling
  • Joints affected
  • –symmetrical
  • –PIP/MCP, wrist, elbow, knee, ankle, MTP
29
Q

What are the examination features of RA?

A 
Palmar erythema
Muscle wasting
Cutaneous vasculitis (digital ischaemia)
Swelling/synovitis
---PIP/MCP, wrist, elbow, knee, ankle, MTP
---Active synovitis = Pain AND swelling
Deformities
-Ulnar devation
-Boutonniere/swan neck
-Z-thumb
30
Q

What are the diagnostic criteria for RA?

A 
American College of Rheumatology criteria
Score >6 for definite RA
A Joint involvement
-2-10 large joints = 1
-1-3 small joints =2, 4-10 small = 3
->10 = 5

B Serology - Low +ve RF/CCP =2, high +ve = 3

C Acute phase response (CRP/ESR) = 1

D Duration >6 weeks = 1

31
Q

What surgical options are there for RA?

A 
Swanson's arthroplasty (silicon joint replacement)
Wrist arthrodesis
Ulnar styloidectomy
Wrist synovectomy
Tendon transfer
32
Q

Extra-articular manifestations of RA

A

Eye: Sjogrens/secondary Sicca, Epi/scleritis, corneal ulcers, cataracts (steroids)

Neuro:
Entrapment Mononeuropathies
Cervical myleoradiculopathy
Mononeuritis multiplex in RA vasculitis

CVS - IHD, pericarditis, MR

Resp:

  1. Fibrosis
  2. Effusion (exudate)
  3. Rheumatoid Nodule
  4. Bronchiolitis obliterans
  5. Pleurisy
  6. Caplans syndrome (silicosis in RA = massive nodules)

Renal - amyloid nephrotic syndrome

Skin - palmar erythema, livedo reticularis, vasculitis, pyoderma gangrenosum

Haem:
Anaemia - chronic disease, drugs (NSAID/MTX)
Leucopenia in felty syndrome

Vasculitis - small vessel, restricted to skin, digital gangrene, raynaud’s

33
Q

Investigations in RA

A

XR of affected joints
MRI/US - sensitive for synovitis
Serology - RhF (Ab to Fc portion of IgG), CCP, CRP/ESR
Joint aspiration (if other causes suspected)

34
Q

Management of RA

A

MDT: OT/physio/CNS/smoking/weight loss

Principles:

  • Aggressive with 2-3 DMARD therapy then wean down
  • MTX and sulfasalazine most common
  • Hydroxychloroquine, leflunomide, ciclosporin, gold, penicillamine

Biologics

  • TNF alfa: Adalimumab, etanercept, infliximab,
  • B cell depletion: rituximab
35
Q

What are the clinical features of Marfan syndrome?

A

Head:

  • Dolichocephaly
  • High arched palate
  • Dental crowding
  • Ectopic lens

Arms:

  • Arm span>height
  • Arachnodactyly
  • Hypermobility

Body:

  • Kyphoscoliosis
  • Pectus deformity
  • Aortic root dilatation/dissection/AR
  • MV prolapse
  • Pneumothorax
36
Q

What is the pathophysiology of Marfan syndrome?

A

AD inherited genetic disorder
-Fibrillin gene mutation

Decreased elastin in tissues and fragmentation of elastic fibres

MRCP PACES revision (8 decks)

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