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Alex's MRCP Cards > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Causes of gout?

A

DECREASED URATE EXCRETION
Drugs
Chronic kidney disease
Lead toxicity

INCREASED URATE PRODUCTION
Myeloproliferative/lymphoproliferative disorder
Cytotoxic drugs
Severe psoriasis

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2
Q

Lesch Nyhan syndrome?

A

Hypoxanthine-guanine phosphoribosyl transferase
deficiency

Inheritance = X-linked recessive

Features: gout, renal failure, learning difficulties,
head-banging

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3
Q

Drug causes of gout?

A

Thiazides, furosemide

Alcohol

Cytotoxic agents

Pyrazinamide

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4
Q

When to start allopurinol?

A

NEW GUIDANCE IS AFTER FIRST ATTACK

Tophi

Renal disease

Uric acid renal stones

Prophylaxis if on cytotoxics or diuretics

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5
Q

Starting allopurinol?

A

Initial dose of 100 mg od, with the dose titrated every few weeks to aim for a serum uric acid of
< 300 μmol/l

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6
Q

What foods are high in purines? (gout)

A

Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast
products

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7
Q

What causes pseudogout?

A

microcrystal synovitis caused by the deposition of calcium pyrophosphate
dihydrate in the synovium

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8
Q

Features of pseudogout?

A

Knee, wrist and shoulders most commonly affected

X-ray: chondrocalcinosis (linear calcification of the articular cartilage)

Joint aspiration: weakly-positively birefringent rhomboid shaped crystals

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9
Q

Risk factors for pseudogout?

A

Hyperparathyroidism

Hypothyroidism

Hemochromatosis

Acromegaly

Low magnesium, Low phosphate

Wilson’s disease

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10
Q

Criteria for RA diagnosis?

A 
1. Morning stiffness > 1 hr (for at
least 6 weeks)
2. Soft-tissue swelling of 3 or
more joints (for at least 6
weeks)
3. Swelling of PIP, MCP or wrist
joints (for at least 6 weeks)
4. Symmetrical arthritis
5. Subcutaneous nodules
6. Rheumatoid factor positive
7. Radiographic evidence of
erosions or periarticular
osteopenia
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11
Q

Antibody for RA?

A

Anti-CCP

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12
Q

X-ray findings in RA?

A

EARLY

  • Loss of joint space (seen in both RA and osteoarthritis)
  • Juxta-articular osteoporosis
  • Soft-tissue swelling

LATE

  • Periarticular erosions (osteopenia and osteoporosis)
  • Subluxation
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13
Q

Poor prognostic features in RA?

A

Rheumatoid factor positive

Poor functional status at presentation

HLA DR4

X-ray: early erosions (in < 2 years)

Extra articular features e.g. Nodules

Female sex

Insidious onset

Anti-CCP antibodies

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14
Q

Extra-articular complications of RA?

A

Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans,
methotrexate pneumonitis, pleurisy

Ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration,
keratitis, steroid-induced cataracts, chloroquine retinopathy

Osteoporosis

IHD: RA carries a similar risk to T2DM

Increased risk of infections

Depression

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15
Q

DMARDs in RA?

A

Methotrexate

Sulfasalazine

Hydroxychloroquine

Leflunomide

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16
Q

Monitoring and complications methotrexate?

A

Monitoring FBC & LFTs is essential due to the

risk of myelosuppression and liver cirrhosis. Other important side-effects include pneumonitis

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17
Q

Indication for TNF inhibitors in RA?

A

inadequate response to at least two DMARDs

including methotrexate

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18
Q

TNF inhibitors in RA?

A

Etanercept: subcutaneous administration, can cause demyelination

Infliximab: intravenous administration, risks include reactivation of
tuberculosis

Adalimumab: subcutaneous administration

STOP 2-4 WEEKS BEFORE SURGERY

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19
Q

Two other types of drugs in RA (not DMARDs or TNF inhibitors)

A

RITUXIMAB
Anti-CD20 monoclonal antibody, results in B-cell depletion
Two 1g intravenous infusions are given two weeks apart
Infusion reactions are common

ABATACEPT
Fusion protein that modulates a key signal required for activation of T lymphocytes
Leads to reduced T-cell proliferation and cytokine production
Given as an infusion

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20
Q

Features of Adult Still’s Disease?

A

Arthralgia

Fever (noticeable at afternoon and evening)

Elevated serum ferritin

Rash: salmon-pink, maculopapular, pruritic

Lymphadenopathy

RF and ANA negative (but ANA 25% positive). Raised ESR and CRP

Leukocytosis and thrombocytosis

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21
Q

Age Adult still’s disease affects?

A

16-35

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22
Q

Organisms causing post-dysenteric reactive arthritis?

A

Shigella flexneri

Salmonella typhimurium

Salmonella enteritidis

Yersinia enterocolitica

Campylobacter

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23
Q

Organism causing post-STI reactive arthritis

A

Chlamydia trachomatis

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24
Q

Antibodies for palindromic arthritis?

A

Anti CCP, AKA

20-50 yrs

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25
Q

Cause of ‘recent-onset arthritis’’? Where does it affect? Blood test?

A

Parvovirus (exposure to kids with febrile illness)

Small hands joints, wrists, elbows, hips, knees, and feet are each affected in > 50% of cases

IgM - detection indicates recent infection, likely parvo

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26
Q

‘A’s of ank spond?

A

Apical fibrosis (CXR)

Anterior uveitis

Aortic regurgitation

Achilles tendonitis

AV node block

Amyloidosis

And cauda equina syndrome

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27
Q

Examinations in ank spond?

A

Reduced lateral flexion (earliest sign), forward flexion and chest expansion

SCHOBER’S TEST - line 10cm above and 5cm below back dimples, distance should increase >5cm in forward flexion

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28
Q

Investigations in ank spond?

A

X RAYS
Sacroilitis: subchondral erosions, sclerosis
Squaring of lumbar vertebrae
‘Bamboo spine’ (late & uncommon)

CXR
Apical fibrosis

SPIRO
Restrictive defect - fibrosis, kyphosis and ankylosis of costovertebral joints

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29
Q

Management of ank spond?

A

NSAIDs

Physiotherapy

Sulphasalazine may be useful if there is peripheral joint involvement - doesn’t improve spinal
mobility

TNF blockers such as etanercept and adalimumab

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30
Q

Seronegative spondyloarthropathies

A

Ankylosing spondylitis

Psoriatic arthritis

Reiter’s syndrome (including reactive arthritis)

Enteropathic arthritis (associated with IBD)

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31
Q

Pseudoxanthoma elasticum?

A

Autosomal recessive condition - abnormality in elastic fibres

Retinal angioid streaks

‘Plucked chicken skin’ appearance - small yellow papules on the neck, antecubital fossa and axillae

Cardiac: mitral valve prolapse, increased risk of ischemic heart disease

Gastrointestinal hemorrhage

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32
Q

Immunology SLE?

A

ANA positive (99% SENSITIVE)

20% are rheumatoid factor positive

Anti-dsDNA: HIGHLY SPECIFIC (> 99%), but less sensitive (70%)

Anti-Smith: MOST SPECIFIC (> 99%), sensitivity (30%)

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33
Q

Complement factor deficiency –> increased SLE risk

A

C3 and C4

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34
Q

HLA in SLE?

A

HLA B8
HLA DR2
HLA DR3

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35
Q

Monitoring in SLE

A

ESR: during active disease the CRP is characteristically normal - a raised CRP may indicate
underlying infection

Complement levels (C3, C4) are low during active disease (formation of complexes leads to
consumption of complement) Low C4 is early marker for disease activity

Anti-DsDNA titers: used for disease monitoring disease activity (but not present in all patients)

36
Q

SLE in pregancy?

A

Worse during prego and puerprium

Can cause neonatal LE

Complications = congenital heart block (anti-Ro)

37
Q

Caues of drug induced lupus?

A

Anti-epileptics: phenytoin

Chlorpromazine

Hydralazine

Isoniazid

Minocycline

Procainamide

38
Q

Features of drug induced lupus?

A

Arthralgia

Myalgia

Skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common

ANA positive in 100%, dsDNA negative

Anti-Ro, anti-Smith positive in around 5%

39
Q

Discoid lupus?

A

Younger females

Rarely progresses to SLE

Follicular keratin plugs

Erythematous raised, scaly rash; face, neck, ears, scalp

Heal with atrophy, scarring, pigmentation

Topical steroid cream, oral antimalarials (hydroxychloroquine), avoid sun exposure

40
Q

Takayasu disease?

A

continuous or patchy granulomatous inflammatory process
involving macrophages, lymphocytes, and multinucleated giant cells which cause progressive occlusive
disease of the aorta and its branches

Rare in western world

41
Q

Presentation of Takayasu disease?

A

Women, 30 years

Fever, malaise, and weight loss;
neurological symptoms such as transient ischemic attacks; or vascular symptoms such as
claudication.

Cardiac - angina, heart failure, and aortic regurgitation.

Renal - mesangial proliferative glomerulonephritis

42
Q

Most specific antibody in dermatomyositis?

A

Anti-Mi-2

43
Q

What is PAN? What is it associated with?

A

vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation

more common in middle-aged men
and is associated with hepatitis B infection

44
Q

Features of PAN?

A

Fever, malaise, arthralgia

Hypertension

Mononeuritis multiplex, sensorimotor polyneuropathy

Hematuria, renal failure

Testicular pain

Abdominal pain (e.g. From mesenteric ischemia)

Perinuclear-antineutrophil cytoplasmic antibodies (pANCA) are found in around 20% of
patients with ‘classic’ PAN

45
Q

Diagnostic criteria for PAN?

A
  1. Weight loss > 4.5 kg.
  2. Livedo reticularis
  3. Testicular pain or tenderness. (Occasionally, a site biopsied for diagnosis).
  4. Muscle pain, weakness, or leg tenderness.
  5. Nerve disease (either single or multiple).
  6. Diastolic BP > 90mmHg
  7. Raised urea/creatinine
  8. Hepatitis B positive (surface antigen or antibody).
  9. Abnormal arteriogram (angiogram)
  10. Biopsy of small/medium size artery (typically inflamed arteries).
46
Q

Summary of vasculitides?

A

Large arteries - GCA, Takayasu’s arteritis

Medium arteries - PAN, Kawasaki disease

Small - Microscopic polyangitis, Wegener’s, Churg Strauss

Arterioles - HSP, Essential cryobulinaemic vasculitis, Anti-GBM antibody mediated disease

47
Q

Features of Wegener’s Granulomatosis?

A

Upper respiratory tract: epistaxis, sinusitis, nasal crusting

Lower respiratory tract: dyspnea,
hemoptysis

Glomerulonephritis
•
Saddle-shape nose deformity

Also: vasculitic rash, eye
involvement (e.g. Proptosis), cranial
nerve lesions

48
Q

Ix and Mx of Wegener’s?

A

Ix - cANCA +ve in >90%, CXR - sometimes cavitating lesions

Mx - Steroids, cyclophosphamide (90% response), plasma exchange

Median survival = 8-9 years

49
Q

Features of Churg-Strauss syndrome?

A

ASTHMA + EOSINOPHILIA + NERVE LESION

Asthma, pulmonary esoinophilic infiltrate
Blood eosinophilia
Paranasal sinusitis
Mononeuritis complex

pANCA +ve in 60% - Anti myeloperoxidase antibody

50
Q

What can sometimes precipitate Churg-Strauss syndrome?

A

Leukotreine receptor antagonists

51
Q

What is HSP

A

IgA mediated small vessel vasculitis

Usually seen in children after infection

Some overlap with IgA nephropathy (Buerger’s disease)

52
Q

Features of HSP?

A

Palpable purpuric rash (with localized edema) over buttocks, extensor surfaces of arms and legs

Abdominal pain, non-bloody diarrhea.

Polyarthritis

Features of IgA nephropathy may occur e.g. Hematuria, renal failure

53
Q

What is thromboangiitis obliterans? (Buerger’s disease)

A

Small/medium vessel vasculitis –> inflammation and ulceration

No CA involvement

Occurs only in cigarette smokers

Presents with arterial ischaemia, progresses proximally, digit gangrene

Treatment is supportive, stop smoking

54
Q

Bechet’s syndrome triad?

A

oral ulcers, genital ulcers and anterior uveitis

Thrombophlebitis
Arthritis
Neurological involvement (e.g. Aseptic
meningitis)
GI: abdo pain, diarrhea, colitis
Erythema nodosum, DVT
55
Q

Random stuff about Bechet’s?

A

Eastern mediterranean (Turkey)

Men > Women, 20-40 yeears

HLA B51, MICA6

Pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule)

Manage with steroids and azathioprine

56
Q

Antiphospholipid syndrome important point?

A

(paradoxically) prolonged APTT + low platelets

Can be primary, or secondary to SLE

57
Q

Features of antiphospholipid syndrome

A

Venous/arterial thrombosis

Recurrent fetal loss

Livedo reticularis

Thrombocytopenia

Prolonged APTT

Other features: pre-eclampsia, pulmonary hypertension

IN PREGNANCY - recurrent miscarriage, IUGR, pre-eclampsia, placental abruption, pre-term delivery, VTE

58
Q

Management of antiphospholipid syndrome? and in pregnancy?

A

Initial VTE - warfarin (2-3) for 6 months

Recurrent VTE - lifelong warfarin (2-3, if on warfarin already then 3-4)

Arterial thrombus - lifelong warfarin (2-3)

PREGNANCY
Low-dose aspirin should be commenced once the pregnancy is confirmed on urine testing

LMWH once a fetal heart is seen on ultrasound. This is usually
discontinued at 34 weeks gestation

59
Q

Features of Raynaud’s suggesting ulderlying CTD?

A

Onset after 40 years

Unilateral symptoms

Rashes

Presence of autoantibodies
•
Digital ulcers, calcinosis

Very rarely: chilblains (pernio) are itchy, painful
purple swellings which occur on the fingers and
toes after exposure to the cold. They are
occasionally associated with underlying connective tissue disease but this is rare

Recurrent miscarriages: This indicates SLE or antiphospholipid syndrome.

60
Q

Secondary causes of Raynaud’s?

A

•
Connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE with
bilateral symptoms

Leukemia

Type I cryoglobulinemia, cold agglutinins

Use of vibrating tools

Drugs: oral contraceptive pill, ergot

Cervical rib

61
Q

Management of Raynaud’s symptoms?

A

Calcium channel blockers

IV prostacyclin infusions

62
Q

Morphea?

A

Localised scleroderma

Small, violaceous or erythematous skin lesions –> enlarge to firm with hypo/hyper-pigmentation

Settle into waxy, white appearance with subsequent atrophy

Resolves within 3-5 years, can last up to 25

ANA rarely positive in localised scleroderma, in systemic subtypes it is always +ve

63
Q

Psoriatic arthropathy?

A

Often precedes skin lesions - 10% with skin lesions develop arthropathy (M = F)

  • Rheumatoid like polyarthritis (most common)
  • Asymmetrical oligoarthritis (hands and feet)
  • Sacroilitis
  • DIP joint disease
  • Arthritis mutilans (severe deformed fingers/hand - ‘telescoping’)
64
Q

Management of Psoriatic arthropathy?

A

Tx as RA - but better prognosis

65
Q

3 types of systemic sclerosis?

A
  1. Limited cutaneous
  2. Diffuse cutaneous
  3. Scleroderma
66
Q

Limited cutaneous systemic sclerosis?

A

Raynaud’s first sign

Scleroderma in face and distal limbs mainly

ANTI-CENTROMERE ANTIBODIES

CREST SYNDROME

67
Q

CREST syndrome?

A

Type of limited cuatneous systemic sclerosis

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangectasia

Malabsopriton secondary to bacterial overgrowth in sclerosed small intestine

Pulmonary HTN is late complication

68
Q

Diffuse cutaneous systemic sclerosis?

A

Affects trunk and proximal limbs mainly

Anti SCL-70 antibodies

HTN, lung fibrosis, renal involvement

Poor prognosis

69
Q

Scleroderma?

A

Without internal organ involvement

Tightening and fibrosis of skin

70
Q

Antibodies for 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

71
Q

Management of systemic sclerosis?

A

Topical treatment for skin changes do not alter the disease course, but may improve pain and
ulceration.

NSAIDs

Limited benefit from steroids

Raynaud’s - nifedipine or other calcium channel
blockers. Dual endothelin-receptor antagonist (bosentan) may be beneficial.

Severe digital ulceration may respond to prostacyclin analogue iloprost

The skin tightness may be treated systemically with methotrexate and ciclosporin

Scleroderma renal crisis: ACE-I to control BP and delay progression to CRF

Active alveolitis is often treated with pulses of cyclophosphamide, often together with a small
dose of steroids

72
Q

Dermatomyositis/Polymyositis?

A

Inflammatory disorder causing symmetrical, proximal
muscle weakness and characteristic skin lesions

Idiopathic, or associated with CTD or malignancy

Polymyositis = variant where skin manifestations not as prominent

73
Q

Dermatomyositis features?

A 
SKIN
Photosensitive
Macular rash over back and shoulder
Heliotrope rash over cheek
Gottron's papules - roughened red papules over extensor
surfaces of fingers
Nail fold capillary dilatation
OTHER
Proximal muscle weakness +/- tenderness
Raynaud's
Respiratory muscle weakness
Interstitial lung disease: e.g. Fibrosing alveolitis or organizing pneumonia
Dysphagia, dysphonia
74
Q

Ix in dermatomyositis?

A

Raised CK

EMG

Muscle biopsy

Anti-jo-1 not common in dermatomyositis, more common in polymyositis

ANA +ve 60%

Screen for malignancy

75
Q

Familial mediterranean fever?

A

Recurrent polyserositis

Autosomal recessive - presents by second decade

Turkish/Armenian, Arabic descnet

76
Q

Features of familial mediterranean fever?

A

Attacks typically last 1-3 days

Pyrexia (on-off)

Constipation/Diarrhea may occur with or after the fever

Abdominal pain (due to peritonitis) – many times appendectomy scar is seen due to multiple
admissions due to abdominal pain

Pleurisy

Pericarditis

Arthritis

Nephrotic syndrome due to renal amyloidosis (that might even need transplantation)

Erysipeloid rash on lower limbs

77
Q

Mx of FMF?

A

Attacks self-limiting, analgesia

Colchicine may reduce attack frequency

78
Q

Relapsing polychondritis?

A

inflammatory condition that involves cartilaginous structures, predominantly those of the pinna, nasal septum and larynx

M + F, fifth decade

79
Q

Features of relapsing polychondritis?

A

Fever, weight loss

sudden onsent ear pain, low hearing

mono/polyarthritis, back pain, myalgia

Mild epistaxis, saddle shaped nose

Redness of eyes (conjunctivitis, episclerits or scleritis)

Hoarse voice, recurrent resp infections

80
Q

Mx of relapsing polychondritis?

A

(no specific test except raised ESR, CRP)

Steroids

Ix for other autoimmune diseases

81
Q

Features of Sjogren’s syndrome?

A

Dry eyes: keratoconjunctivitis sicca

Dry mouth

Vaginal dryness

Arthralgia

Raynaud’s, myalgia

Sensory polyneuropathy

Renal tubular acidosis (usually
subclinical)

AT INCREASED RISK OF LYMPHOID MALIGNANCY (40-60 fold)

82
Q

Ix in Sjogren’s?

A

Rheumatoid factor (RF) positive in nearly 100% of patients

ANA positive in 70%

Anti-Ro (SSA) antibodies in 70% of patients with PSS

Anti-La (SSB) antibodies in 30% of patients with PSS

Schirmer’s test: filter paper near conjunctival sac to measure
tear formation

Histology: focal lymphocytic infiltration (increased risk malignancy)

Also: hypergammaglobulinemia, low C4

83
Q

Secondary Sjogren’s?

A

RA or other CTD (SLE) - develops about 10 years after onset

84
Q

Causes avascular necrosis of hip? Ix?

A

long-term steroid use
chemotherapy
alcohol excess
trauma

Plain X-ray normal initially
MRI = gold standard

85
Q

Mnemonic for SLE?

A

MD SOAP BRAIN

M alar rash
D iscoid rash

S erositis - pleurisy, pericarditis, pericardial/pleural effusions
O ral ulcers
A rthralgia - mainly peripheral joints
P hotosensitivity

B lood disorder - Pancytopenia, or individual cytopenia
R enal - SLE nephritis
A NA+
I mmunological markers - Anti-dsDNA, Anti-Sm, Antiphospholipid syndrome
N europsychiatrical: Psychosis, Seizures.

86
Q

Paget’s disease Ix?

A

ALP raised, calcium phosphate normal

Other markers of bone turnover - procollagen type I N-terminal propeptide (PINP), serum C-telopeptide (CTx), urinary N-telopeptide (NTx), and urinary hydroxyproline

87
Q

McArdle’s syndrome?

A

autosomal recessive type V glycogen storage disease
caused by myophosphorylase deficiency
this causes decreased muscle glycogenolysis

  • muscle pain and stiffness following exercise
  • muscle cramps
  • myoglobinuria
  • low lactate levels during exercise

Alex's MRCP Cards (13 decks)

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