rheumatology Flashcards

Question

how is dermatomyositis managed?

Answer 1

Prednisolone

Answer 2

Pseudoxanthoma elasticum is an inherited condition (usually autosomal recessive*) characterised by an abnormality in elastic fibres

Answer 3

retinal angioid streaks 'plucked chicken skin' appearance - small yellow papules on the neck, antecubital fossa and axillae cardiac: mitral valve prolapse, increased risk of ischaemic heart disease gastrointestinal haemorrhage

Answer 4

Marfan's is an AD connective tissue disorder It is caused by a defect in the FBN1 gene on chromosome 15 that codes for protein fibrillin-1. It affects around 1 in 3000 people?

Answer 5

tall stature with arm span to height ratio > 1.05 high-arched palate arachnodactyly pectus excavatum pes planus scoliosis of > 20 degrees heart: > dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation > mitral valve prolapse (75%), lungs: repeated pneumothoraces eyes: > upwards lens dislocation (superotemporal ectopia lentis) > blue sclera > myopia dural ectasia (ballooning of the dural sac at the lumbosacral level)

Answer 6

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 > 450 µmol/l)

Answer 7

Staph. aureus is the most common cause except in patients with sickle-cell anaemia where Salmonella species predominate

Answer 8

ULT is particularly recommended if: >= 2 attacks in 12 months tophi renal disease uric acid renal stones prophylaxis if on cytotoxics or diuretics

Answer 9

NSAIDs Colchicine oral steroids if NSAIDs and colchicine are contraindicated * if patient is already taking allopurinol it should continued Urate lowering therapy - allopurinol 1st line 2nd line when allopurinol is not tolerated or ineffective is febuxostat (also a xanthine oxidase inhibitor) In refractory cases other agent may be tired - uricase - pegolticase

Answer 10

reduce alcohol intake and avoid during an acute attack lose weight if obese avoid food high in purines e.g. Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast products consideration should be given to stopping precipitating drugs (such as thiazides) losartan has a specific uricosuric action and may be particularly suitable for the many patients who have coexistent hypertension increased vitamin C intake (either supplements or through normal diet) may also decrease serum uric acid levels

Answer 11

Azathioprine, mesalazine and sulfasalazine (with folic acid) are safe to use in pregnancy and breastfeeding.

Answer 12

Azathioprine is metabolised to the active compound mercaptopurine, a purine analogue that inhibits purine synthesis. A thiopurine methyltransferase (TPMT) test may be needed to look for individuals prone to azathioprine toxicity.

Answer 13

bone marrow depression - consider a full blood count if infection/bleeding occurs nausea/vomiting pancreatitis increased risk of non-melanoma skin cancer

Answer 14

Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

Answer 15

Limited cutaneous systemic sclerosis Raynaud's may be the first sign scleroderma affects face and distal limbs predominately associated with anti-centromere antibodies a subtype of limited systemic sclerosis is CREST syndrome: Calcinosis, Raynaud's phenomenon, oEsophageal dysmotility, Sclerodactyly, Telangiectasia Diffuse cutaneous systemic sclerosis scleroderma affects trunk and proximal limbs predominately associated with anti scl-70 antibodies the most common cause of death is now respiratory involvement, which is seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) other complications include renal disease and hypertension patients with renal disease should be started on an ACE inhibitor poor prognosis Scleroderma (without internal organ involvement) tightening and fibrosis of skin may be manifest as plaques (morphoea) or linear

Answer 16

ANA positive in 90% RF positive in 30% anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis associated with a higher risk of severe interstitial lung disease anti-centromere antibodies associated with limited cutaneous systemic sclerosis

Answer 17

respiratory involvement: interstitial lung disease and pulmonary arterial hypertension

Answer 18

a rare disorder characterised by the proliferation of Langerhans cells, which are specialised dendritic cells that normally function to present antigen to T lymphocytes. The disease can affect multiple organs, including the bones, skin, lungs, and endocrine system. It is notable for its variable clinical presentation, ranging from isolated bone lesions to multisystem disease.

Answer 19

bone pain, typically in the skull or proximal femur cutaneous nodules pituitary involvement: leads to diabetes insipidus due to pituitary stalk involvement pulmonary involvement: More common in adults, presenting with dyspnoea, cough, and chest pain recurrent otitis media/mastoiditis tennis racket-shaped Birbeck granules on electromicroscopy

Answer 20

biopsy: confirmation is through biopsy showing Langerhans cells with characteristic grooved nuclei and positive staining for CD1a and S100 protein imaging: radiographs and MRI for bone lesions; CT may be used for chest and abdominal involvement

Answer 21

localized disease: surgical resection or limited radiotherapy for isolated lesions. multisystem disease: systemic therapy including steroids, chemotherapy (e.g., vinblastine, cytarabine), and targeted therapies for refractory cases. supportive care: management of diabetes insipidus, pain control, and treatment of secondary infections.

Answer 22

Adhesive capsulitis: external rotation Rotator cuff: Abduction, Adduction, medial and lateral rotation

Answer 23

Pilocarpine is a cholinergic agonist that stimulates muscarinic receptors to increase saliva production.

Answer 24

A significant interaction may occur with allopurinol and hence lower doses of azathioprine should be used.

Answer 25

Azathioprine is generally considered safe to use in pregnancy.

Answer 26

Equivalent of prednisolone 7.5 mg or more each day for 3 months.

Answer 27

Lateral femoral cutaneous nerve (LFCN)

Answer 28

Entrapment mononeuropathy of the LFCN

Answer 29

Iatrogenic causes, surgical procedures, or neuromas

Answer 30

L2/3 segments

Answer 31

Sensory nerve, carrying no motor fibres

Answer 32

Passes behind the psoas muscle, runs beneath the iliac fascia, exits through or under the inguinal ligament

Answer 33

Around the anterior superior iliac spine (ASIS) ## Footnote Compression of this nerve anywhere along its course can lead to the development of meralgia paraesthetica.

Answer 34

People aged between 30 and 40

Answer 35

More common in men

Answer 36

Those with diabetes

Answer 37

* Obesity * Pregnancy * Tense ascites * Trauma * Iatrogenic causes * Various sports activities

Answer 38

* Burning * Tingling * Coldness * Shooting pain * Numbness * Deep muscle ache

Answer 39

Aggravated by standing, relieved by sitting

Answer 40

Deep palpation just below the ASIS (pelvic compression)

Answer 41

Altered sensation over the upper lateral aspect of the thigh

Answer 42

Pelvic compression test

Answer 43

Abolishes the pain

Answer 44

Nerve conduction studies

Answer 45

Key features are tenderness maximal at a point 5cm distal to the lateral epicondyle, and the pain is worse on forearm pronation. Pronation of the forearm can increase tension in the radial tunnel and exacerbate nerve compression - a finding characteristic of radial tunnel syndrome.

Answer 46

Lateral epicondylitis typically follows unaccustomed activity such as house painting or playing tennis ('tennis elbow'). It is most common in people aged 45-55 years and typically affects the dominant arm.

Answer 47

pain and tenderness localised to the lateral epicondyle pain worse on wrist extension against resistance with the elbow extended or supination of the forearm with the elbow extended episodes typically last between 6 months and 2 years. Patients tend to have acute pain for 6-12 weeks

Answer 48

advice on avoiding muscle overload simple analgesia steroid injection physiotherapy

Answer 49

Golfer's elbow pain and tenderness localised to the medial epicondyle pain is aggravated by wrist flexion and pronation symptoms may be accompanied by numbness / tingling in the 4th and 5th finger due to ulnar nerve involvement

Answer 50

Due to the compression of the ulnar nerve. Features initially intermittent tingling in the 4th and 5th finger may be worse when the elbow is resting on a firm surface or flexed for extended periods later numbness in the 4th and 5th finger with associated weakness

Answer 51

Swelling over the posterior aspect of the elbow. There may be associated pain, warmth and erythema. It typically affects middle-aged male patients.

Answer 52

Methotrexate needs to be stopped 6 months before concepion leflunomide is not safe in pregnancy sulfasalazine and hydroxychloroquine are considered safe in pregnancy Low dose steroids may be used to control symptoms NSAIDs may be used until 32 weeks but after this time should be withdrawn due to the risk of early close of the ductus arteriosus patients should be referred to an obstetric anaesthetist due to the risk of atlanto-axial subluxation

Answer 53

In drug-induced lupus not all the typical features of systemic lupus erythematosus are seen, with renal and nervous system involvement being unusual. It usually resolves on stopping the drug. Features arthralgia myalgia skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common ANA positive in 100%, dsDNA negative anti-histone antibodies are found in 80-90% anti-Ro, anti-Smith positive in around 5%

Answer 54

Most common causes procainamide hydralazine Less common causes isoniazid minocycline phenytoin

Answer 55

Acrocyanosis is characterised by persistent cyanosis of the extremities, usually in response to cold exposure. It involves a bluish discolouration of the hands, feet, or face without the pain, numbness, or the episodic nature seen in Raynaud’s phenomenon.

Answer 56

Raynaud's phenomenon is characterised by an exaggerated vasoconstrictive response of the digital arteries and cutaneous arteriole to the cold or emotional stress. It may be primary (Raynaud's disease) or secondary (Raynaud's phenomenon). Raynaud's disease typically presents in young women (e.g. 30 years old) with bilateral symptoms.

Answer 57

* connective tissue disorders scleroderma (most common) rheumatoid arthritis systemic lupus erythematosus * leukaemia * type I cryoglobulinaemia, cold agglutinins * use of vibrating tools * drugs: oral contraceptive pill, ergot * cervical rib

Answer 58

onset after 40 years unilateral symptoms rashes presence of autoantibodies features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages digital ulcers, calcinosis very rarely: chilblains

Answer 59

all patients with suspected secondary Raynaud's phenomenon should be referred to secondary care first-line: calcium channel blockers e.g. nifedipine IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months

Answer 60

fatigue fever mouth ulcers lymphadenopathy

Answer 61

malar (butterfly) rash: spares nasolabial folds discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas. Lesions may progress to become pigmented and hyperkeratotic before becoming atrophic photosensitivity Raynaud's phenomenon livedo reticularis non-scarring alopecia

Answer 62

arthralgia non-erosive arthritis

Answer 63

pericarditis: the most common cardiac manifestation myocarditis

Answer 64

pleurisy fibrosing alveolitis

Answer 65

proteinuria glomerulonephritis (diffuse proliferative glomerulonephritis is the most common type)

Answer 66

anxiety and depression psychosis seizures

Answer 67

Compression of median nerve in the carpal tunnel

Answer 68

Pain/pins and needles in thumb, index, middle finger ## Footnote Symptoms may 'ascend' proximally and patients often shake their hands for relief, especially at night.

Answer 69

Patient shakes his hand to obtain relief, classically at night

Answer 70

Weakness of thumb abduction (abductor pollicis brevis)

Answer 71

Wasting of thenar eminence (NOT hypothenar)

Answer 72

Tapping causes paraesthesia

Answer 73

Flexion of wrist causes symptoms

Answer 74

Idiopathic Pregnancy Oedema (e.g. heart failure) Lunate fracture Rheumatoid arthritis

Answer 75

Prolongation of the action potential

Answer 76

NICE recommends a 6-week trial of conservative treatments if the symptoms are mild-moderate corticosteroid injection wrist splints at night: particularly useful if transient factors present e.g. pregnancy if there are severe symptoms or symptoms persist with conservative management: surgical decompression (flexor retinaculum division)

Answer 77

**Cubital tunnel syndrome occurs due to compression of the ulnar nerve as it passes through the cubital tunnel. Tingling and numbness of the 4th and 5th finger which starts off intermittent and then becomes constant. Over time patients may also develop weakness and muscle wasting Pain worse on leaning on the affected elbow Often a history of osteoarthritis or prior trauma to the area. ## Footnote Investigations the diagnosis is usually clinical however, in selected cases nerve conduction studies may be used

Answer 78

Avoid aggravating activity Physiotherapy Steroid injections Surgery in resistant cases

Answer 79

Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia. It may occur as a primary disorder or secondary to other conditions, most commonly systemic lupus erythematosus (SLE). Around 30% of patients with SLE have positive antiphospholipid antibodies. A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade.

Answer 80

venous/arterial thrombosis recurrent miscarriages livedo reticularis other features: pre-eclampsia, pulmonary hypertension

Answer 81

** anticardiolipin antibodies anti-beta2 glycoprotein I (anti-beta2GPI) antibodies lupus anticoagulant** thrombocytopenia prolonged APTT

Answer 82

Clinical criteria (at least one must be met): ≥1 clinical episode of arterial, venous, or small-vessel thrombosis (confirmed by imaging or histopathology) Pregnancy morbidity: three or more consecutive, unexplained spontaneous abortions <10 weeks, or ≥1 unexplained fetal loss ≥10 weeks, or ≥1 preterm birth <34 weeks due to placental insufficiency Laboratory criteria (at least one must be met on ≥2 occasions at least 12 weeks apart): Lupus anticoagulant (LA) Anticardiolipin (aCL) antibodies Anti-beta2 glycoprotein I (anti-β2GP1) antibodies

Answer 83

Primary thromobopropylaxis - low dose aspirin, consider LMWH Secondary thromboprophylaxis - lifelong warfarin INR 2-3 recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin, increase target INR to 3-4 arterial thrombosis should be treated with lifelong warfarin with target INR 2-3 Pregnancy - low dose aspirin with LMWH

Answer 84

Lupus anticogulant

Answer 85

Ottawa rules An ankle x-ray is required only if there is any pain in the malleolar zone and any one of the following findings: bony tenderness at the lateral malleolar zone (from the tip of the lateral malleolus to include the lower 6 cm of posterior border of the fibular) bony tenderness at the medial malleolar zone (from the tip of the medial malleolus to the lower 6 cm of the posterior border of the tibia) -inability to walk four weight-bearing steps immediately after the injury and in the emergency department

Answer 86

Basics NSAIDs sun-block Hydroxychloroquine the treatment of choice for SLE If internal organ involvement e.g. renal, neuro, eye then consider prednisolone, cyclophosphamide

Answer 87

Early x-ray findings loss of joint space juxta-articular osteoporosis soft-tissue swelling periarticular erosions subluxation

Answer 88

Osteoarthritis *- Joint space narrowing, osteophytes, subchondral sclerosis, and subchondral cysts. Rheumatoid arthritis * Early x-ray findings: loss of joint space, juxta-articular osteoporosis, soft-tissue swelling * Late x-ray findings: periarticular erosions, subluxation Gout * Soft tissue swelling, punched-out bone lesions and overhanging sclerotic margins. Ankylosing spondylitis * Sacroiliitis: subchondral erosions, sclerosis * - Syndesmophytes: due to ossification of outer fibers of annulus fibrosus * - Squaring of lumbar vertebrae * - 'Bamboo spine' (late & uncommon) * - Chest x-ray: apical fibrosis Psoriatic arthritis * - Often have the unusual combination of coexistence of erosive changes and new bone formation * - Periostitis * - 'Pencil-in-cup' appearance

Answer 89

Sulfasalazine is a disease modifying anti-rheumatic drug (DMARDs) used in the management of inflammatory arthritis, especially rheumatoid arthritis. It is also used in the management of inflammatory bowel disease.

Answer 90

Sulfasalazine is a prodrug for 5-ASA which works through decreasing neutrophil chemotaxis alongside suppressing proliferation of lymphocytes and pro-inflammatory cytokines.

Answer 91

Cautions G6PD deficiency allergy to aspirin or sulphonamides (cross-sensitivity)

Answer 92

oligospermia Stevens-Johnson syndrome pneumonitis / lung fibrosis myelosuppression, Heinz body anaemia, megaloblastic anaemia may colour tears → stained contact lenses ## Footnote In contrast to other DMARDs, sulfasalazine is considered safe to use in both pregnancy and breastfeeding.

Answer 93

Avascular necrosis (AVN) may be defined as death of bone tissue secondary to loss of the blood supply. This leads to bone destruction and loss of joint function. It most commonly affects the epiphysis of long bones such as the femur.

Answer 94

long-term steroid use chemotherapy alcohol excess trauma

Answer 95

initially asymptomatic pain in the affected joint

Answer 96

plain x-ray findings may be normal initially osteopenia and microfractures may be seen early on collapse of the articular surface may result in the crescent sign MRI is the investigation of choice it is more sensitive than radionuclide bone scanning

Answer 97

Basics T score: based on bone mass of young reference population T score of -1.0 means bone mass of one standard deviation below that of young reference population Z score is adjusted for age, gender and ethnic factors T score > -1.0 = normal -1.0 to -2.5 = osteopaenia < -2.5 = osteoporosis

Answer 98

Lumbar spinal stenosis is a condition in which the central canal is narrowed by tumour, disk prolapse or other similar degenerative changes.

Answer 99

Patients may present with a combination of back pain, neuropathic pain and symptoms mimicking claudication. One of the main features that may help to differentiate it from true claudication in the history is the positional element to the pain. Sitting is better than standing and patients may find it easier to walk uphill rather than downhill. The neurogenic claudication type history makes lumbar spinal stenosis a likely underlying diagnosis, the absence of such symptoms makes it far less likely.

Answer 100

Degenerative disease is the commonest underlying cause. Degeneration is believed to begin in the intervertebral disk where biochemical changes such as cell death and loss of proteoglycan and water content lead to progressive disk bulging and collapse. This process leads to an increased stress transfer to the posterior facet joints, which accelerates cartilaginous degeneration, hypertrophy, and osteophyte formation; this is associated with thickening and distortion of the ligamentum flavum. The combination of the ventral disk bulging, osteophyte formation at the dorsal facet, and ligamentum flavum hyptertrophy combine to circumferentially narrow the spinal canal and the space available for the neural elements. The compression of the nerve roots of the cauda equina leads to the characteristic clinical signs and symptoms of lumbar spinal stenosis.

Answer 101

Diagnosis MRI scanning is the best modality for demonstrating the canal narrowing. Historically a bicycle test was used as true vascular claudicants could not complete the test. Treatment Laminectomy

Answer 102

Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder which typically presents by the second decade. It is more common in people of Turkish, Armenian and Arabic descent.

Answer 103

Features - attacks typically last 1-3 days pyrexia abdominal pain (due to peritonitis) pleurisy pericarditis arthritis erysipeloid rash on lower limbs Management colchicine may help

Answer 104

Anterior ischemic optic neuropathy - fundoscopy typically shows a swollen pale disc and blurred margins

Answer 105

fundoscopy would show dilated, tortuous veins, 'cotton wool spots' and 'flame haemorrhages'.

Answer 106

Discoid lupus erythematosus is a benign disorder generally seen in younger females. It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases). Discoid lupus erythematosus is characterised by follicular keratin plugs and is thought to be autoimmune in aetiology

Answer 107

erythematous, raised rash, sometimes scaly may be photosensitive more common on face, neck, ears and scalp lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation

Answer 108

topical steroid cream oral antimalarials may be used second-line e.g. hydroxychloroquine avoid sun exposure

Answer 109

Leflunomide is a disease modifying anti-rheumatic drug (DMARD) mainly used in the management of rheumatoid arthritis. It has a very long half-life which should be remembered considering it's teratogenic potential.

Answer 110

pregnancy - the BNF advises: 'Effective contraception essential during treatment and for at least 2 years after treatment in women and at least 3 months after treatment in men (plasma concentration monitoring required' caution should also be exercised with pre-existing lung and liver disease

Answer 111

gastrointestinal, especially diarrhoea hypertension weight loss/anorexia peripheral neuropathy myelosuppression pneumonitis

Answer 112

Monitoring FBC/LFT and blood pressure Stopping leflunomide has a very long wash-out period of up to a year which requires co-administration of cholestyramine