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MRCP PACES revision > Rheumatology > Flashcards

Rheumatology Flashcards

(36 cards)

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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

Study These Flashcards
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

Study These Flashcards
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

Study These Flashcards
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

Study These Flashcards
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?

Study These Flashcards
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

Study These Flashcards
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?

Study These Flashcards
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?

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

How are the viscera effected in sclerosis?

Study These Flashcards
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
How is systemic sclerosis managed?
``` MDT approach - refer to appropriate specialties Immunomodulation: -Steroids (esp. pulmonary fibrosis) -Cyclophosphamide -MMF ```
26
How is Raynaud's managed?
Non-pharmacological: - Hand warmers - Warm clothing - Primrose oil Vasodilators - Calcium channel blockers - Losartan - Topical GTN Severe (ulcers/necrosis): -Iloprost
27
How is a scleroderma renal crisis managed?
1. ACEi (70 --> 10% mortality) | 2. RRT
28
What are the symptoms of rheumatoid arthritis?
- Weeks to months onset - Stiffness - worse in morning/inactivity - Pain - Swelling - Joints affected - --symmetrical - --PIP/MCP, wrist, elbow, knee, ankle, MTP
29
What are the examination features of RA?
``` 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
What are the diagnostic criteria for RA?
``` 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
What surgical options are there for RA?
``` Swanson's arthroplasty (silicon joint replacement) Wrist arthrodesis Ulnar styloidectomy Wrist synovectomy Tendon transfer ```
32
Extra-articular manifestations of RA
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
Investigations in RA
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
Management of RA
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
What are the clinical features of Marfan syndrome?
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
What is the pathophysiology of Marfan syndrome?
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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