MED: Rheumatology Flashcards

Question

What are the clinical features of PAN?

Answer 1

- fever, malaise, arthralgia - weight loss - hypertension - mononeuritis multiplex, sensorimotor polyneuropathy - testicular pain - livedo reticularis - haematuria, renal failure - p-ANCA are found in around 20% of patients with 'classic' PAN - hepatitis B serology positive in 30% of patients

Answer 2

**Laboratory testing:** *Basic laboratory tests help ascertain the extent of involvement of different organs:* - LFTs - Hepatitis B and C serologies - Creatinine kinase - Serum creatinine - Urine analysis - ESR and CRP are significantly elevated **Chest radiography:** - May be obtained to exclude other forms of vasculitis, which have greater involvement in the lungs. **Biopsy:** - Performed on a clinically affected organ to confirm the diagnosis. **Imaging:** - Arteriography (mesenteric or renal) can be used as an alternative to biopsy to confirm the diagnosis (to minimise bleeding risk). It can reveal aneurysms and irregular constrictions in the vessels. - CT/MRI can demonstrate wedge shaped renal infarctions, which are less specific than microaneurysms showed on arteriography.

Answer 3

**Mild disease with constitutional symptoms but no end-organ damage:** - Oral prednisone (1mg/kg body weight). **Mild disease but resistant to or intolerant to of required dose of glucocorticoids:** - Addition of azathioprine or methotrexate to a tolerable dose of glucocorticoids. - Methotrexate should be avoided in patients with renal disease or hepatitis. **Moderate to severe disease, with evidence of end-organ damage:** - High-dose glucocorticoids and cyclophosphamide, followed by azathioprine or methotrexate for remission maintenance. - In patients with mild PAN and associated hepatitis B or C infection, it is advised to treat initially with antivirals only, and not to give any immunosuppressants. - However, patients with severe hepatitis virus-associated PAN may be benefited from short term glucocorticoids or plasma exchange until antiviral therapy becomes effective.

Answer 4

An inflammatory condition that causes pain and stiffness in the shoulder and pelvic girdles

Answer 5

*Subacute onset (2-6 week history) of:* - Shoulder and/or pelvic girdle pain and stiffness lasting at least 45 minutes (may be initially unilateral but then becomes bilateral). - Shoulder girdle pain - can be accompanied by tenderness, signs of bursitis and restriction of shoulder movement (e.g. difficulty raising their arms to brush their hair or getting out of bed). Patients may also localise pain and tenderness to the neck muscles. - Pelvic girdle pain - tenderness and signs of bursitis may be observed and, on observation, patients may have difficulty rising from a chair. - Improvement in symptoms in one week with a trial of corticosteroids - Systemic symptoms - low grade fever, fatigue, anorexia, weight loss and depression. - Peripheral oligoarticular arthritis - wrists, knees and metacarpophalangeal joints may be affected at presentation >>If the patient is younger than 50, an alternative explanation for the above symptoms should be sought as PMR is very rare in this age group.

Answer 6

- <60yrs - Red flags of serious pathology (including weight loss, night pain or neurological features) but do not have any other identifiable cause for these - Do not have the core symptoms of PMR (bilateral shoulder/pelvic girdle pain, >45 minutes of morning stiffness) - Have unusual features of PMR (normal, or very high inflammatory markers, chronic onset of symptoms, limited response to steroids) >>If patients present with PMR present with symptoms of GCA (headache, jaw claudication, visual disturbance) then urgent referral should be sought.

Answer 7

**Baseline blood tests:** - CRP/ESR - both are usually is elevated at diagnosis. *The following would be expected to be normal in PMR but raised in the conditions listed:* - FBC - infections, leukaemia - U&E - myeloma, renal failure - LFT - infections, metastatic disease - TSH - hypothyroidism - Calcium - hyperparathyroidism, metastatic disease - Creatine kinase - polymyositis, statin-induced myopathy. - Serum protein electrophoresis - myeloma - RF and anti-CCP - rheumatoid arthritis - Other cancer biomarkers (e.g. PSA, CA-125) if there are features in the history suggestive of these conditions. **Additional tests recommended include:** - Urine dip - looking for haematuria as suggestive of cancer. - Urinary Bence Jones protein - myeloma - Chest x-ray - lung cancer, also needed as a baseline prior to considering methotrexate, which may be used in secondary care. **Imaging:** - Rarely needed and would rarely be requested in primary care. However, if the diagnosis is unclear, USS can be helpful to screen for bursitis with trochanteric bursitis being the most common bursitis and subacromial bursitis being associated with PMR.

Answer 8

- Age >50 years - Acute or subacute onset of symptoms representative of PMR - Response to corticosteroids (prednisolone) - Acute phase response - elevated CRP or ESR - Exclusion of other conditions that mimic PMR - active infection, active cancer and GCA

Answer 9

**Primary care management:** - Oral corticosteroids - Gradually weaned off them with dose adjustments typically being every 4-8 weeks and reviews (telephone or face to face) scheduled for one week after each dose adjustment. - Safety netting about the signs and symptoms of GCA and ensure patients know to seek urgent medical care if these develop. **Secondary care management:** - Patients should be referred if they have frequent relapses or are at higher risk of adverse effects from corticosteroid treatment - Patients may be considered for DMARD treatment as 2nd line therapy (e.g. methotrexate) or tocilizumab as 3rd line.

Answer 10

haemochromatosis hyperparathyroidism acromegaly low magnesium, low phosphate Wilson's disease

Answer 11

- knee, wrist and shoulders most commonly affected - joint aspiration: weakly-positively birefringent rhomboid-shaped crystals

Answer 12

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

Answer 13

- Aspiration of joint fluid, to exclude septic arthritis - NSAIDs or intra-articular, IM or oral steroids as for gout

Answer 14

A chronic inflammatory arthritis which is associated with psoriasis. It may be develop alongside the rash seen in psoriasis, or years later. In rare cases it can be seen without a psoriatic rash.

Answer 15

**Psoriasis:** - Current or past psoriatic rash or nail changes - Rash/nail changes usually precede joint involvement. - Nail changes may be subtle, and can include pitting, ridges or onycholysis. **Inflammatory arthritis:** - Joint pain which is worse after long periods of rest, features prolonged morning stiffness of >30 minutes, and improves with activity. - O/E signs of synovitis (tenderness and swelling of joints). **Features specific to psoriatic arthritis:** - Dactylitis (swelling of a whole digit) - NICE advise referral of anyone with dactylitis to a rheumatologist for assessment - Enthesitis (inflammation at the site of tendon attachment) - commonly presents as elbow, heel or lateral hip pain - Patients may report history of achilles tendonitis, plantar fasciitis or epicondylitis

Answer 16

>>Diagnosis often clinical >>Diagnosis and management should be coordinated by secondary care, so referral to a rheumatologist should be made if psoriatic arthritis is suspected. *Investigations can be used to support diagnosis, or to help in differentiating between different forms of arthritis* **Blood tests:** - Inflammatory markers (ESR/CRP) - often raised, especially in active disease - RF - non-specific, so may be positive in up to 10% patients with psoriatic arthritis - Anti-CCP - may be positive, more likely in severe disease - HLA-B27 - positive result increases the probability of spondyloarthritis **Imaging:** - Plain film x-rays - changes may be absent in early disease, DIP joint erosion and periarticular new-bone formation, osteolysis and pencil-in-cup deformity in advanced disease/arthritis mutilans - Ultrasound - helpful for diagnosis in absence of x-ray changes, tendon swelling, increased blood flow and erosions suggest inflammation - MRI of affected joints may be considered >>If psoriatic arthritis is confirmed, sacroiliac x-rays should be offered to look for evidence of asymptomatic involvement.

Answer 17

**Conservative:** - Physiotherapy - Hand therapy - Podiatry - Occupational therapy >>Aim to assess their needs, provide advice/equipment, and regular follow-up **Medical:** - NSAIDs (NICE recommend using as adjunct to DMARDs) - Intra-articular corticosteroid injections - alone or in combination with oral medication - DMARDs considered if there is a failure of response to initial medical treatment or if there is severe disease at diagnosis - 1st line: standard DMARDs (methotrexate, leflunomide or sulfasalazine) - 2nd line: biological agents (etanercept, infliximab, apremilast) - Short courses of oral corticosteroids may be needed while initiating DMARD therapy

Answer 18

Triphasic colour change of the affected extremities: - Pallor (white): This phase results from the initial vasoconstriction of arterioles, leading to reduced blood flow. - Cyanosis (blue): As the oxygen supply in the tissues diminishes, a bluish discolouration occurs. - Hyperemia (red): During the recovery phase, vasodilation leads to increased blood flow, causing a reddish hue. Accompanied by symptoms: - Numbness - Cold sensation - Tingling - Pain >>Episodes can last from minutes to hours and typically resolve upon rewarming or cessation of the triggering factor.

Answer 19

>>Essential to differentiate between primary and secondary Raynaud's to guide treatment and management. - FBC, ESR, CRP - ANA - RF and anti-CCP antibodies - Cold-stimulus test - Nailfold capillaroscopy - Imaging studies, such as Doppler ultrasonography, to evaluate for occlusive arterial disease.

Answer 20

**Non-pharmacological interventions:** - Patient education: Inform patients about the importance of avoiding triggers, such as cold exposure and emotional stress. - Temperature regulation: Encourage patients to wear gloves, socks, and appropriate clothing to maintain warmth in cold environments. - Smoking cessation: Smoking can exacerbate symptoms by causing vasoconstriction. - Stress management: Teach patients stress reduction techniques, such as deep breathing exercises, meditation, and progressive muscle relaxation. **Pharmacological interventions:** - First-line = CCB e.g. nifedipine or amlodipine - Second line = Alpha-blockers e.g. Prazosin and doxazosin - Severe/refractory = Vasodilators e.g. Sildenafil, tadalafil, and prostaglandin analogs (e.g., iloprost) **Surgical interventions:** - Sympathectomy: In severe or refractory cases, a surgical sympathectomy may be performed to interrupt the sympathetic nerve supply to the affected extremity, promoting vasodilation and reducing vasospastic episodes. This approach should be reserved for cases that do not respond to conservative measures and pharmacological therapy. - Revascularization procedures: In cases of secondary Raynaud's with arterial occlusive disease, surgical or endovascular revascularization may be required to restore blood flow to the affected extremity.

Answer 21

Usually 1-6w following gastrointestinal (Salmonella, Shigella Campylobacter) or urogenital pathogens (Chlamydia trachomatis)

Answer 22

**MSK manifestations:** - Peripheral arthritis - acute asymmetric oligoarthritis (usually causing knee, ankle, and/or heel pain), Painful, swollen, warm, red, and stiff joints, especially in the morning - Dactylitis (sausage digit) - painful inflammation of an entire finger or toe - Enthesitis - inflammation of ligaments and tendons at the sites where they insert into bones, common sites are Achilles tendonitis presenting heel pain, plantar fasciitis, and pain at the tibial tubercle - Axial arthritis -spinal inflammation, especially of the sacroiliac joints and lumbosacral spine, manifests as nonspecific low back pain and/or buttock pain and stiffness, especially during times of inactivity. **Extra‑articular manifestations:** - Ocular inflammation in the form of conjunctivitis can cause redness, tearing, and a sterile purulent discharge during the acute period. - Anterior uveitis - pain, redness, and photophobia. - Keratitis, corneal ulceration, episcleritis, retrobulbar neuritis, and anterior chamber haemorrhage may be present in chronic disease. - Chlamydia infection cause genitourinary symptoms in 70-80% patients. - In men, prostatitis is found in 80% of cases. But, urethritis and hemorrhagic cystitis can also occur. - In women, non‑purulent cervicitis causing cervical bleeding, salpingo-oophoritis, or cystitis can occur. - Yersinia infection causes mild diarrhoea - Salmonella and Campylobacter infections can cause severe and of longer duration diarrhoea increasing the likeliness to develop ReA - Colonoscopic findings in these patients resemble UC or CD - Keratoderma blennorhagicum: hyperkeratotic skin on palms and soles which mimics pustular psoriasis - Circinate balanitis: painless ulcers and plaque-like lesions on the shaft or glans of the penis - Oral ulcers - Nail changes like onycholysis, subungual keratosis, or nail pits - Constitutional symptoms: malaise, fever, fatigue, and weight loss are - Cardiac manifestations: pericarditis, aortic disease, conduction abnormalities

Answer 23

>>Clinical diagnosis **Blood tests:** - Elevated ESR and CRP - RF and ANA - rule out other forms of arthritis - HLA‑B27 testing: non-diagnostic test, but positive in around 40% of patients. It is useful as a prognostic marker of ReA. **Evidence of preceding infection:** - Urinalysis may show increased leukocytes, hematuria, and mild proteinuria during acute disease. - NAAT to detect Chlamydia trachomatis - Stool test - Salmonella, Shigella, Campylobacter, and Yersinia **Evidence of inflammatory arthritis, enthesitis or synovitis:** - Radiography - in the early stage, there are no specific abnormalities except for soft tissue swelling. - Radiographic changes are found in 70% of patients with long‑standing disease (chronic ReA). - Erosive joint damage affects especially small joints of the feet - Radiographic changes of sacroiliac joints, usually unilateral - MRI: Helpful to assess enthesitis and involvement of sacroiliac joints, when radiographs may be negative **Synovial fluid analysis:** - Joint fluid (when possible) should always be examined to exclude alternative diagnoses like septic arthritis. - Synovial fluid cell count is increased, with predominant neutrophils in the early stage of the disease. - Synovial cultures are always negative.

Answer 24

**Treatment of arthritis:** - NSAIDs: first-line therapy for acute phase - Corticosteroids - during an acute flare, or unresponsive to NSAIDs. - Intra-articular injections in mono-articular and oligoarticular forms, while systemic corticosteroids may be required if many joints are affected. - DMARDs - second-line agents either when NSAIDs and CS fails or for those who develop chronic or erosive reactive arthritis - Sulfasalazine is effective in peripheral disease and has little or no effect on spinal disease. - Methotrexate is effective in treating both acute and chronic ReA especially, in patients with spinal involvement. - Anti-TNF-α therapy - if symptoms persist after all other treatments exhausted. Etanercept is an emerging therapy, effective in treating chronic ReA on a small series of cases. - Physiotherapy - may help relieve symptoms by increasing muscle strength and improving mobility and function **Antibiotics:** - Acute Chlamydia genitourinary infection or with previously documented but untreated infection, but not for patients with established reactive arthritis - Antibiotics are generally not indicated for uncomplicated gastrointestinal infections. - Oral regimens of either doxycycline or azithromycin, both combined with rifampin for 6 months have shown significant symptom remission **Treatment of other manifestations:** - Circinate balanitis and mild to moderate keratoderma blennorrhagica should be treated with topical steroids. - Oral lesions resolve spontaneously and require no treatment. - Eye lesions, especially uveitis, should be managed with ophthalmologic advice. - Systemic corticosteroids can be helpful in treating ocular manifestations, such as anterior uveitis.

Answer 25

- Symmetrical Joint Involvement - polyarthritis, with joint pain, swelling, and stiffness - Small joints of hands and feet, e.g. MCP, PIP, and MTP joints predominantly affected. - Larger joints, including the knees, shoulders, and elbows, can also be involved. - Morning Stiffness lasting >30 minutes - may improve with activity. - Stiffness can also occur after periods of inactivity - 'gelling.' - Fatigue - attributed to the chronic inflammatory state, anaemia of chronic disease, sleep disturbances, or medication side effects. - Systemic symptoms - low-grade fever, weight loss, and general malaise - Insidious onset, with symptoms developing over weeks to months (some patients may present with a more acute onset) - Disease course ranges from intermittent flares to persistent, unremitting inflammation

Answer 26

- Rheumatoid nodules - Interstitial lung disease - Keratoconjunctivitis sicca - Anemia of chronic disease

Answer 27

**Bloods:** - Rheumatoid factor - Anti-CCP antibody **X-ray:** *Early x-ray findings* - loss of joint space - juxta-articular osteoporosis / osteopaenia - soft-tissue swelling *Late x-ray findings* - periarticular erosions - subluxation

Answer 28

- High WBC count, predominantly PMNs - Appearance is typically yellow and cloudy - Absence of crystals

Answer 29

Sjogren's syndrome (around 100%) Felty's syndrome (around 100%) infective endocarditis (= 50%) SLE (= 20-30%) systemic sclerosis (= 30%) general population (= 5%) rarely: TB, HBV, EBV, leprosy

Answer 30

**First line:** - DMARD monotherapy + short course prednisolone (to induce remission) - First line DMARD = methotrexate (taken once weekly) - Must monitor FBC and LFTs (risk of myelosuppression and cirrhosis) - Other options = sulfasalazine, leflunomide, hydroxychloroquine **Second line:** *If inadequate response to at least two DMARDs including methotrexate* - TNF-inhibitors - E.g. etanercept, infliximab, adalimumab - Also - rituximab

Answer 31

- 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 - Ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus - Increased risk of infections - Depression *Less common* - Felty's syndrome (RA + splenomegaly + low white cell count) - Amyloidosis

Answer 32

- dry eyes: keratoconjunctivitis sicca - dry mouth - vaginal dryness - arthralgia - Raynaud's, myalgia - sensory polyneuropathy - recurrent episodes of parotitis - renal tubular acidosis (usually subclinical)

Answer 33

- rheumatoid factor (RF) positive in around 50% 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 - also: hypergammaglobulinaemia, low C4

Answer 34

artificial saliva and tears pilocarpine may stimulate saliva production

Answer 35

A multisystemic autoimmune disorder in which autoantibodies form immune complexes

Answer 36

- Malar/ butterfly rash (fixed erythematous rash of the cheek bones that spares the nasolabial folds) - Photosensitivity - Arthralgia - Constitutional sx - fever, weight loss and malaise etc. - Arthritis - Mouth ulcers - Alopecia (non-scarring) - Discoid erythematosus (erythematous raised patches with scaling and follicular plugging) - Lymphadenopathy - Livedo reticularis (mottled reticular patterned skin rash of purple discolouration)

Answer 37

- Renal - Lupus nephritis - Respiratory - Pleuritis, Pneumonitis, PE - Cardiovascular - Pericarditis, Raynaud's, Endocarditis, Atherosclerosis - Neuropsychiatric - Headache or migraine, Seizures, Psychosis - Osteoporosis - Malignancies

Answer 38

Anti-phospholipid syndrome Sjogren's disease Autoimmune thyroid disease

Answer 39

- FBC - normochromic normocytic anaemia, leucopenia, thrombocytopenia - ESR raised, CRP normal - ANA - Anti-dsDNA antibody - highly specific for SLE - Anti-Smith antibody - presence is diagnostic ] - C3 and C4 - decreased levels show active disease / flares - U&Es - deranged in lupus nephritis

Answer 40

**Pharmacological:** - NSAIDs - joint or serosal inflammation - Hydroxychloroquine - all SLE patients unless contraindicated due to its steroid-sparing effect and potential to reduce flares. It also possesses cardioprotective properties. - Glucocorticoids (pred) - for acute flares and severe disease - Immunosuppressants - moderate to severe disease, e.g. methotrexate, azathioprine, mycophenolate mofetil, and cyclophosphamide - Biologics like rituximab, belimumab, and anifrolumab may be used in refractory cases. **Non-Pharmacological:** - Lifestyle modifications - smoking cessation, balanced diet, regular exercise - Sun protection - avoid excessive sun exposure and use sun-protective measures (UV light can trigger flares) - Psychosocial support - support groups, counselling, and CBT **Management of Comorbidities:** - Cardiovascular disease: Regular cardiovascular risk assessment and appropriate management of hypertension, hyperlipidaemia, and diabetes is required. - Infections: Vaccinations against pneumococcus, influenza, and HPV are recommended, and infections should be promptly identified and treated. - Osteoporosis: Bone mineral density should be assessed regularly, and vitamin D and calcium supplementation, along with bisphosphonates, may be required. **Monitoring:** - FBC, renal function, LFTs, complement levels, and anti-dsDNA antibody levels are typical components of monitoring.

Answer 41

// Scleroderma A chronic, autoimmune connective tissue disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation.

Answer 42

**Limited cutaneous systemic sclerosis (lcSSc):** - Scleroderma affects face and distal limbs predominately - Associated with anti-centromere antibodies - A subtype of limited systemic sclerosis is CREST syndrome **Diffuse cutaneous systemic sclerosis (dcSSc):** - Scleroderma affects trunk and proximal limbs predominately - Associated with anti scl-70 antibodies

Answer 43

Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia

Answer 44

Skin: Thickening, tightening, and hardening of the skin, which typically starts in the fingers and progresses proximally. Raynaud's phenomenon: Vasospasm-induced color changes (pallor, cyanosis, and erythema) in the fingers and toes in response to cold or stress. Gastrointestinal involvement: Dysphagia, gastroesophageal reflux, and motility disorders affecting the esophagus, stomach, and intestines. Pulmonary involvement: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are common complications, which can cause shortness of breath, cough, and chest pain. Renal involvement: Scleroderma renal crisis, characterized by rapidly progressive renal failure and malignant hypertension, is a life-threatening complication of SSc. Musculoskeletal involvement: Joint pain, stiffness, and contractures due to fibrosis of the joint capsules and tendons.

Answer 45

**Autoantibodies:** - Anti-topoisomerase I (anti-Scl-70) - diffuse - Anticentromere - limited - Anti-RNA polymerase III **Nailfold capillaroscopy:** - Abnormal capillary patterns, including dilatation, avascular areas, and neoangiogenesis, are suggestive of SSc. **Imaging studies:** - High-resolution CT can help detect ILD - Echo can screen for pulmonary arterial hypertension. **Skin biopsy:** - Although not routinely performed, a skin biopsy showing dermal fibrosis and collagen deposition can provide histopathological evidence of SSc.

Answer 46

- Vasodilator therapy: CCBs, phosphodiesterase-5 inhibitors, and prostacyclin analogues can help manage Raynaud's phenomenon, digital ulcers, PAH - Immunosuppressive therapy: Corticosteroids, methotrexate, mycophenolate mofetil, and cyclophosphamide can be used to reduce inflammation and slow the progression of fibrosis, particularly in cases of ILD and rapidly progressive skin involvement. - GI management: PPIs and prokinetic agents can help manage gastroesophageal reflux and motility disorders, while dietary modifications may be necessary for malabsorption or constipation. - Renal management: Prompt treatment with ACEIs is crucial in the management of scleroderma renal crisis. - PT/OT: Help maintain joint mobility, improve muscle strength, and address functional limitations. - Psychological support

Answer 47

Large vessel vasculitis

Answer 48

- sYstemic features of a vasculitis e.g. malaise, headache - Unequal blood pressure in the upper limbs - Carotid bruit - Intermittent claudication - Aortic regurgitation (around 20%) - absent limb pulse

Answer 49

**Inflammatory markers:** - Both ESR and CRP are likely to be raised **Computerised tomography angiography (CTA):** - The investigation of choice - Magnetic resonance angiography (MRA) may also be used **Ultrasound (+/- doppler enhancement):** - This is more readily available than other imaging methods and is preferred in early or mild disease and children.

Answer 50

**Corticosteroids:** - Oral prednisolone (1mg/kg/day) is usually first line. - Steroids should be given alongside low dose aspirin (75mg daily). **Immunosuppressive agents:** - Considered 2nd line and are utilised when patients relapse during steroid tapering. - Options include: Methotrexate (given with folic acid), Azathioprine, Mycophenolate mofetil, Cyclophosphamide. **Additional considerations:** - Bone protection is required due to the long-term use of steroids - alendronic acid with calcitriol and calcium carbonate - Patients on high dose prednisolone may require PCP prophylaxis with trimethoprim. - If both steroids and immunosuppressive treatments are ineffective, a tumour necrosis factor (TNF)-alpha inhibitor (such as infliximab) may be started. - Surgical intervention is indicated in patients with severe limb claudication or organ dysfunction - percutaneous angioplasty and vascular bypass surgery.

Answer 51

Common: - Hypertension - long term complication which may result either from RAS, or aortic stenosis, or both. - Peripheral vascular ischaemia - stenosis and occlusions of arteries are common. These should be investigated regularly with vascular imaging studies. Less common: - Aortic aneurysm or regurgitation - Angina and congestive heart failure - Cerebrovascular disease - low risk of both stroke and TIA

Answer 52

- Typically patient > 60 years old - Usually rapid onset (e.g. < 1 month) - Headache - Jaw claudication - Temporary / permanent visual loss - amaurosis fugax - Diplopia - Tender, palpable temporal artery - Around 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness) - Also lethargy, depression, low-grade fever, anorexia, night sweats

Answer 53

- Irreversible vision loss - most serious complication, due to anterior ischemic optic neuropathy - Retinal artery occlusion, diplopia, and ptosis - Stroke - inflammation and occlusion of cerebral arteries - Aortic aneurysm and dissection - GCA can affect the aorta, leading to aortitis, which may progress to aortic aneurysm or dissection - Large vessel involvement - subclavian, axillary, and vertebral arteries, leading to limb claudication, upper extremity ischemia, or vertebrobasilar insufficiency. - PMR - up to 50% of patients - Systemic complications - fever, malaise, weight loss, and anaemia. - Long-term use of corticosteroids can result in complications like osteoporosis, diabetes, hypertension, and increased susceptibility to infections

Answer 54

*If 3 or more of the criteria are present below, the patient is highly suspected to have temporal arteritis:* - Age of onset≥50 years - New onset of headache - Temporal artery abnormality: tenderness, thickening or reduced pulsation of temporal artery - High ESR>50mm/hr - Changes consistent with GCA on biopsy

Answer 55

- Raised inflammatory markers - ESR >50, CRP may also be elevated - Temporal artery biopsy - skip lesions may be present - Fundoscopy to check for anterior ischemic optic neuropathy - typically shows a swollen pale disc and blurred margins

Answer 56

- Urgent high-dose glucocorticoids (soon as diagnosis suspected, before temporal artery biopsy) - No visual loss =high-dose prednisolone - Evolving visual loss = IV methylprednisolone given prior to starting high-dose prednisolone - There should be a dramatic response, if not the diagnosis should be reconsidered - Urgent ophthalmology review (patients with visual symptoms should be seen the same-day by an ophthalmologist) *other treatments:* - bone protection with bisphosphonates is required as long, tapering course of steroids is required - low-dose aspirin is sometimes given to patients as well, although the evidence base supporting this is weak

Answer 57

- Usually bilateral - one joint at a time affected over a period of several years - The carpometacarpal joints (CMCs), distal interphalangeal joints (DIPJs) are affected most - Episodic joint pain - intermittent ache provoked by movement and relieved by rest - Stiffness - worse after long periods of inactivity e.g. waking up in the morning // only lasts a few minutes - Painless nodes (bony swellings) - Heberden’s nodes at the DIP joints, Bouchard’s Nodes at the PIP joints (result of osteophyte formation) - Squaring of the thumbs - deformity of the carpometacarpal joint of the thumb resulting in fixed adduction of the thumb.

Answer 58

X-ray changes of osteoarthritis (LOSS) - Loss of joint space - Osteophytes forming at joint margins - Subchondral sclerosis - Subchondral cysts

Answer 59

- All patients offered help with weight loss, given advice about local muscle strengthening exercises and general aerobic fitness - Topical NSAIDs are first-line analgesics (knee / hand) - Second-line treatment is oral NSAIDs (first line for hip) - PPI should be co-prescribed with NSAIDs - Non-pharmacological treatment options include walking aids for knee and hip OA - Intra-articular steroid injections may be tried if standard pharmacological treatment is ineffective - If conservative methods fail then refer for consideration of joint replacement

Answer 60

- **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 If the x-ray is negative for sacroiliac joint involvement in ankylosing spondylitis but suspicion for AS remains high, the next step in the evaluation should be obtaining an MRI. Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis

Answer 61

- Typically presents in males aged 20-30 - Symptoms of inflammatory joint pain (worst in the morning, improve with exercise) - E.g. lower back pain that radiates to buttocks -

Answer 62

- Encourage regular exercise such as swimming - NSAIDs are the first-line treatment - Physiotherapy - 'Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments'

Answer 63

Trimethoprim / co-trimoxazole High-dose aspirin

Answer 64

folic acid 5mg once weekly, taken more than 24 hours after methotrexate dose

Answer 65

**Beighton score** - Positive if at least 5/9 in adults, or at least 6/9 in children

Answer 66

Autosomal dominant connective tissue disorder. Results in tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin

Answer 67

- elastic, fragile skin - joint hypermobility: recurrent joint dislocation - easy bruising - aortic regurgitation, mitral valve prolapse and aortic dissection - subarachnoid haemorrhage - angioid retinal streaks

Answer 68

- Venous and arterial thromboses - Recurrent fetal loss - Thrombocytopenia - Livedo reticularis (mottled discolouration of the skin) CLOTS (clots, livedo reticularis, obstetric complications and thrombocytopenia)

Answer 69

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

Answer 70

**Primary thromboprophylaxis:** - Low-dose aspirin **Secondary thromboprophylaxis:** - Initial venous thromboembolic events / arterial thrombosis = lifelong warfarin with a target INR of 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

Answer 71

Osteomalacia

Answer 72

- Vitamin D deficiency - malabsorption, lack of sunlight, diet - Chronic kidney disease - Drug induced e.g. anticonvulsants - Inherited: hypophosphatemic rickets (previously called vitamin D-resistant rickets) - Liver disease: e.g. cirrhosis - Coeliac disease

Answer 73

bone pain bone/muscle tenderness fractures: especially femoral neck proximal myopathy: may lead to a waddling gait

Answer 74

**Bloods:** - low vitamin D - low calcium, phosphate - raised ALP **X-ray:** - translucent bands (Looser's zones or pseudofractures)

Answer 75

vitamin D supplmentation - loading dose is often needed initially calcium supplementation if dietary calcium is inadequate

Answer 76

Consider an alternative diagnosis Should respond dramatically

Answer 77

Caused by autoantibodies against aminoacyl-tRNA synthetase e.g. anti-Jo1. *It is characterised by:* - myositis - interstitial lung disease - thickened and cracked skin of the hands (mechanic's hands) - Raynaud's phenomenon

Answer 78

Since corticosteroids increase the risk of osteoporosis significantly, anyone that is likely to start steroids for at least 3 months should start bone protection immediately. This is in the form of first correcting hypocalcaemia and vitamin D (25-hydroxycholecalciferol) deficiencies, and then giving bisphosphonates.

Answer 79

- 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 80

advice on avoiding muscle overload simple analgesia steroid injection physiotherapy

Answer 81

Following a fragility fracture in women ≥ 75 years, a DEXA scan is not necessary to diagnose osteoporosis and hence commence a bisphosphonate

Answer 82

Psoriatic arthritis

Answer 83

Oesophageal ulceration

Answer 84

An inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions

Answer 85

- photosensitive - macular rash over back and shoulder - heliotrope rash in the periorbital region - 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

Answer 86

- proximal muscle weakness +/- tenderness - Raynaud's - respiratory muscle weakness - interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia - dysphagia, dysphonia

Answer 87

- Majority of patients ANA positive - Some patients have antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including: antibodies against histidine-tRNA ligase (also called **Jo-1**) antibodies to signal recognition particle (SRP) anti-Mi-2 antibodies 1 in 4 adults with dermatomyositis has an underlying malignancy driving the disease. It is therefore important to fully investigate these patients for malignancy. The easiest and most reliable method of doing this is a CT chest/abdomen/pelvis as it is quick to obtain and has a high specificity for picking up any underlying malignancy.

Answer 88

polymyositis

Answer 89

- mucositis - painful aphthous-like ulcers - myelosuppression - pneumonitis - pulmonary fibrosis - liver fibrosis

Answer 90

- oesophageal reactions - oesophagitis, oesophageal ulcers - 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

Answer 91

1) oral ulcers 2) genital ulcers 3) anterior uveitis

Answer 92

RA, splenomegaly and low white cell count