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MRCP PART 1 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Sjogren’s syndrome has increased risk of what malignancy?

A

Increase in lymphoid malignancy.
Non-Hodgkin’s lymphoma is the most common malignancy in these patients.

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2
Q

Acute management for gout?

A
  • NSAIDs or colchicine are first-line
  • Colchicine inhibits microtubule polymerization by binding to tubulin, interfering with mitosis. Also inhibits neutrophil motility and activity
    has a slower onset of action
    may be used with caution in renal impairment. The main side-effect is diarrhoea
  • Oral steroids may be considered if NSAIDs and colchicine are contraindicated. A dose of prednisolone 15mg/day is usually used.
  • Another option is intra-articular steroid injection.
  • If the patient is already taking allopurinol it should be continued.
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3
Q

Osteogenesis imperfecta is caused by abnormal synthesis of what type of collagen?

A

Osteogenesis imperfecta occurs due to an abnormality in type 1 collagen.

Osteogenesis imperfecta (brittle bone disease) is a group of disorders of collagen metabolism resulting in bone fragility and fractures.

  • Autosomal dominant
  • Abnormality in type 1 collagen due to decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
  • Presents in childhood
  • Fractures following minor trauma
  • Blue sclera
  • Deafness secondary to otosclerosis
  • Dental imperfections
  • Adjusted calcium, phosphate, parathyroid hormone and ALP results are usually normal in osteogenesis imperfecta.
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4
Q

Bloods results seen in Paget’s disease?

A

Raised alkaline phosphatase (ALP)
calcium and phosphate are typically normal. Hypercalcaemia may occasionally occur with prolonged immobilisation.

Paget’s disease is a disease of increased but uncontrolled bone turnover. It is thought to be primarily a disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity.

Remember old man, bone pain, isolated raised ALP

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5
Q

Main adverse effect of hydroxychloroquine?

A
  • Bull’s eye Retinopathy - may result in severe and permanent visual loss.
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6
Q

Treatment for dry mouth in Sjogren’s syndrome?

A
  • Artificial saliva and tears
  • Pilocarpine may stimulate saliva production
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7
Q

Drugs causing drug induced lupus?

A

Most common:
Procainamide
Hydralazine

Less common:
Isoniazid
Minocycline
Phenytoin

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8
Q

Antibody for drug induced lupus?

A

Anti-histone antibodies

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9
Q

Classic triad of Behcet’s syndrome?

A

The classic triad of symptoms are oral ulcers, genital ulcers and anterior uveitis.

Associated with HLA B51.

Also associated with:
- Thrombophlebitis and deep vein thrombosis
- Arthritis
- Neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum

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10
Q

The presence of anti-Ro antibodies in a pregnant patient with SLE leads to an increased risk of what condition in the foetus?

A

Congenital heart block.

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11
Q

Concurrent prescription of which antibiotic is avoided in patient’s taking methotrexate?

A

The concurrent use of methotrexate and trimethoprim containing antibiotics may cause bone marrow suppression and severe or fatal pancytopaenia.

Also co-trimoxazole.

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12
Q

Adverse effects of methotrexate?

A
  • Mucositis
  • Myelosuppression
  • Pneumonitis: most common pulmonary manifestation
    similar disease pattern to hypersensitivity pneumonitis,
    typically develops within a year of starting treatment, presents with non-productive cough, dyspnoea, malaise, fever.
  • Pulmonary fibrosis
  • Liver fibrosis
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13
Q

In lateral epicondylitis tennis elbow, which movement worsens the pain?

A

pain worse on wrist extension against resistance with the elbow extended or supination of the forearm with the elbow extended.

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14
Q

What does joint aspiration show in pseudogout?

A

Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium.

Joint aspiration: weakly-positively birefringent rhomboid-shaped crystals.

X-ray: chondrocalcinosis
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage.

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15
Q

Main eye complication in temporal arteritis?

A

Anterior ischemic optic neuropathy accounts for the majority of ocular complications. It results from occlusion of the posterior ciliary artery (a branch of the ophthalmic artery) → ischaemia of the optic nerve head. Fundoscopy typically shows a swollen pale disc and blurred margins.

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16
Q

Antibody associated with diffuse cutaneous systemic slerosis?

A

Anti-scl-70 antibodies

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17
Q

Antibody associated with limited cutaneous systemic sclerosis?

A

Anti-centromere antibodies

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18
Q

Features of reactive arthritis?

A
  1. Urethritis
  2. Conjunctivitis
  3. Arthritis

Can be following a GI infection or STI.

HLA B27 seronegative spondyloartropathy.

‘Can’t see, pee or climb a tree’

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19
Q

Name of skin rash found in reactive arthritis?

A

Keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles).

20
Q

How long does methotrexate need to be stopped prior to conception?

A

6 months.

21
Q

Adverse effects of bisphosphonates?

A
  • Oesophageal reactions: oesophagitis, oesophageal ulcers (especially alendronate)
  • Osteonecrosis of the jaw
  • increased risk of atypical stress fractures of the proximal femoral shaft in patients taking alendronate
  • Acute phase response: fever, myalgia and arthralgia may occur following administration
  • Hypocalcaemia: due to reduced calcium efflux from bone. Usually clinically unimportant

Tablets should be swallowed whole with plenty of water while sitting or standing; to be given on an empty stomach at least 30 minutes before breakfast (or another oral medication)

22
Q

Drugs used in rheumatology that are safe for prenancy?

A

Drugs safe in Pregnancy: PASH

P: Prednisolone
A: Azathioprine
S: Sulfasalazine
H: Hydroxychloroquine

23
Q

Management for osteoarthritis?

A
  1. Paracetamol and topical NSAIDs are first-line analgesics. Topical NSAIDs are indicated only for OA of the knee or hand.
  2. Second-line treatment is oral NSAIDs/COX-2 inhibitors, opioids, capsaicin cream and intra-articular corticosteroids. A proton pump inhibitor should be co-prescribed with NSAIDs and COX-2 inhibitors (e.g. celecoxib). These drugs should be avoided if the patient takes aspirin.

If conservative methods fail then refer for consideration of joint replacement

24
Q

What are the clinical features of De Quervain’s tenosynovitis?

A

Common condition in which the sheath containing the extensor pollicis brevis and abductor pollicis longus tendons is inflamed. Typically affects females aged 30 - 50.

Features:
- Pain on the radial side of the wrist
- Tenderness over the radial styloid process
- Abduction of the thumb against resistance is painful

Finkelstein’s test: the examiner pulls the thumb of the patient in ulnar deviation and longitudinal traction. In a patient with tenosynovitis this action causes pain over the radial styloid process and along the length of extensor pollisis brevis and abductor pollicis longus.

Question stem will sometimes mention woman with young children from carrying them often.

25
Q

Antibody for Antiphospholipid syndrome?

A

Anticardiolipin antibody is one of three tests that support a diagnosis of antiphospholipid syndrome.

The others are lupus anticoagulant and anti-beta2-glycoprotein.

There is also prolonged APTT and thrombocytopenia.

CLOTS
Clotting (arterial and venous)
Livedo reticularis
Obstetric (miscarriages)
Thrombocytopaenia

26
Q

Drug causes of gout?

A

Drug causes:

  • Diuretics: thiazides, furosemide, indapamide, chlortalidone
  • Ciclosporin
  • Alcohol
  • Cytotoxic agents
  • Pyrazinamide
  • Aspirin
27
Q

What causes gout?

A

Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium. It is caused by chronic hyperuricaemia (uric acid > 0.45 mmol/l).

28
Q

Features of ankylosing spondylitis?

A

The ‘A’s:
- Apical fibrosis
- Anterior uveitis
- Aortic regurgitation
- Achilles tendonitis
- AV node block
- Amyloidosis

and cauda equina syndrome
peripheral arthritis

29
Q

Marfan’s syndrome is caused by mutation in a gene that encodes what protein?

A

Fibrillin-1.

Marfan’s syndrome is an autosomal dominant connective tissue disorder. It is caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1.

30
Q

What is the mechanism of action of Mycophenolate?

A

Mycophenolate mofetil is an immunosuppressant commonly used to prevent rejection of organ transplants.

  • Inhibits inosine monophosphate dehydrogenase, which is needed for purine synthesis.
  • As T and B cells are particularly dependent on this pathway it can reduce the proliferation of immune cells
31
Q

Which antibody is most specific for SLE?

A

Anti Smith antibodies.

Anti-Smith (Sm) antibodies are highly specific for SLE being present in approximately 30% of patients with this condition. They are directed against proteins found in the cell nucleus and although not all SLE patients will have them, their presence is almost exclusive to SLE, making them a highly specific marker.

32
Q

Low levels of which complement are associated with the development of SLE?

A

Complement levels (C3, C4) are low during active disease (formation of complexes leads to consumption of complement).

33
Q

Antibodies seen in SLE?

A

99% are ANA positive:
this high sensitivity makes it a useful rule out test, but it has low specificity

20% are Rheumatoid Factor positive
- Anti-dsDNA: highly specific (> 99%), but less sensitive (70%)

  • Anti-Smith: highly specific (> 99%), sensitivity (30%)
  • Anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
34
Q

Presence of which antibody supports the diagnosis of Mixed connective tissue disease?

A

It is associated with anti-U1 ribonucleoprotein (RNP) antibodies.

Persistent high titres of anti-ribonuclear protein (anti-RNP) are considered a necessary diagnostic criterion for MCTD. Although it can be present in other autoimmune diseases (e.g. SLE), by the main current diagnostic criteria it is always present in patients with mixed connective tissue disease.

35
Q

What are the blood results seen in osteomalacia?

A
  • Low calcium
  • Low phosphate
  • Raised PTH
  • Raised alkaline phosphatase
36
Q

Which antibodies are seen in antiphospholipid syndrome?
List 3

A
  • Anticardiolipin antibodies
  • Anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
  • Lupus anticoagulant
37
Q

What are the x-ray findings for ankylosing spondylitis?

A

Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis.

Radiographs may be normal early in disease, later changes include:
sacroiliitis: subchondral erosions, sclerosis.
- squaring of lumbar vertebrae
- ‘bamboo spine’ (late & uncommon)
- syndesmophytes: due to ossification of outer fibers of annulus fibrosus
- chest x-ray: apical fibrosis

38
Q

Proximal muscle weakness and a heliotrope rash is seen in which condition?

A

Dermatomyositis.

Polymyositis and dermatomyositis are autoimmune disorders where there is inflammation in the muscles (myositis). Polymyositis is a condition of chronic inflammation of muscles. Dermatomyositis is a connective tissue disorder where there is a chronic inflammation of the skin and muscles. The presence of cutaneous manifestations favours a diagnosis of dermatomyositis.

39
Q

What are the skin features seen in Dermatomyositis?

A
  • Macular rash over back and shoulder (Shawl sign).
  • Heliotrope rash in the periorbital region (lilac discolouration of the eyelids with swelling)
  • Gottron’s papules - roughened red papules over extensor surfaces of fingers.
  • ‘Mechanic’s hands’: extremely dry and scaly hands with linear ‘cracks’ on the palmar and lateral aspects of the fingers
  • Nail fold capillary dilatation
40
Q

Management for Paget’s disease?

A
  • Bisphosphonate (either oral risedronate or IV zoledronate)
  • Calcitonin is less commonly used now
41
Q

Which enzyme does MTX inhibit?

A

Methotrexate is an antimetabolite that inhibits Dihydrofolate Reductase, an enzyme essential for the synthesis of purines and pyrimidines.

42
Q

Antibody most specific for dermatomyositis?

A

Anti-Mi-2 Antibodies.

(Anti-Jo-1 antibodies are more specific for polymyositis.)

43
Q

Management for Raynaud’s including second line drug treatment ?

A

First-line: calcium channel blockers e.g. Nifedipine

2nd line: IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

44
Q

Ehlers Danlos is commonly associated with defect in which collagen type?

A

Ehlers-Danlos syndrome is primarily caused by a genetic defect in collagen type III. Collagen Type V is affected in a less common variant of Ehlers-Danlos syndrome.

45
Q

What is the drug of choice for long term management of SLE?

A

Hydroxychloroquine is considered to be the ‘anchor drug’ for long term maintenance therapy of SLE and all patients should be commenced on this treatment at diagnosis.

46
Q

Behcet syndrome is associated with what HLA?

A

Behcet’s syndrome is associated with HLA B51.

MRCP PART 1 (20 decks)

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