Rheumatology

Question 1

Sjogren’s syndrome has increased risk of what malignancy?

Answer 1

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

Question 2

Acute management for gout?

Answer 2

• 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.

Question 3

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

Answer 3

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.

Question 4

Bloods results seen in Paget’s disease?

Answer 4

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

Question 5

Main adverse effect of hydroxychloroquine?

Answer 5

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

Question 6

Treatment for dry mouth in Sjogren’s syndrome?

Answer 6

• Artificial saliva and tears
• Pilocarpine may stimulate saliva production

Question 7

Drugs causing drug induced lupus?

Answer 7

Most common:
Procainamide
Hydralazine

Less common:
Isoniazid
Minocycline
Phenytoin

Question 8

Antibody for drug induced lupus?

Answer 8

Anti-histone antibodies

Question 9

Classic triad of Behcet’s syndrome?

Answer 9

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

Question 10

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

Answer 10

Congenital heart block.

Question 11

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

Answer 11

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

Also co-trimoxazole.

Question 12

Adverse effects of methotrexate?

Answer 12

• 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

Question 13

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

Answer 13

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

Question 14

What does joint aspiration show in pseudogout?

Answer 14

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.

Question 15

Main eye complication in temporal arteritis?

Answer 15

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.

Question 16

Antibody associated with diffuse cutaneous systemic slerosis?

Answer 16

Anti-scl-70 antibodies

Question 17

Antibody associated with limited cutaneous systemic sclerosis?

Answer 17

Anti-centromere antibodies

Question 18

Features of reactive arthritis?

Answer 18

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’

Question 19

Name of skin rash found in reactive arthritis?

Answer 19

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

Question 20

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

Answer 20

6 months.

Question 21

Adverse effects of bisphosphonates?

Answer 21

• 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)

Question 22

Drugs used in rheumatology that are safe for prenancy?

Answer 22

Drugs safe in Pregnancy: PASH

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

Question 23

Management for osteoarthritis?

Answer 23

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

Question 24

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

Answer 24

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.

Question 25

Antibody for Antiphospholipid syndrome?

Answer 25

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

Question 26

Drug causes of gout?

Answer 26

Drug causes: - Diuretics: thiazides, furosemide, indapamide, chlortalidone - Ciclosporin - Alcohol - Cytotoxic agents - Pyrazinamide - Aspirin

Question 27

What causes gout?

Answer 27

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).

Question 28

Features of ankylosing spondylitis?

Answer 28

The 'A's: - Apical fibrosis - Anterior uveitis - Aortic regurgitation - Achilles tendonitis - AV node block - Amyloidosis and cauda equina syndrome peripheral arthritis

Question 29

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

Answer 29

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.

Question 30

What is the mechanism of action of Mycophenolate?

Answer 30

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

Question 31

Which antibody is most specific for SLE?

Answer 31

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.

Question 32

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

Answer 32

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

Question 33

Antibodies seen in SLE?

Answer 33

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)

Question 34

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

Answer 34

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.

Question 35

What are the blood results seen in osteomalacia?

Answer 35

- Low calcium - Low phosphate - Raised PTH - Raised alkaline phosphatase

Question 36

Which antibodies are seen in antiphospholipid syndrome? List 3

Answer 36

- Anticardiolipin antibodies - Anti-beta2 glycoprotein I (anti-beta2GPI) antibodies - Lupus anticoagulant

Question 37

What are the x-ray findings for ankylosing spondylitis?

Answer 37

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

Question 38

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

Answer 38

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.

Question 39

What are the skin features seen in Dermatomyositis?

Answer 39

- 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

Question 40

Management for Paget's disease?

Answer 40

- Bisphosphonate (either oral risedronate or IV zoledronate) - Calcitonin is less commonly used now

Question 41

Which enzyme does MTX inhibit?

Answer 41

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

Question 42

Antibody most specific for dermatomyositis?

Answer 42

Anti-Mi-2 Antibodies. (Anti-Jo-1 antibodies are more specific for polymyositis.)

Question 43

Management for Raynaud's including second line drug treatment ?

Answer 43

First-line: calcium channel blockers e.g. Nifedipine 2nd line: IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

Question 44

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

Answer 44

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.

Question 45

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

Answer 45

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.

Question 46

Behcet syndrome is associated with what HLA?

Answer 46

Behcet's syndrome is associated with HLA B51.