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  • Question 1 - A 30-year-old woman with a history of rheumatoid arthritis seeks advice on starting...

    Incorrect

    • A 30-year-old woman with a history of rheumatoid arthritis seeks advice on starting a family with her partner. Her rheumatoid arthritis is currently well-managed with methotrexate and sulphasalazine, and she has not needed to adjust her doses for the past two years. However, she is hesitant to stop taking her medications unless it is necessary, as she experienced several flares when her doses were reduced three years ago. What recommendations would you provide regarding her pregnancy plans?

      Your Answer: She should reconsider her plans for pregnancy. Stopping medications would make her disease uncontrollable and continuing medications will affect her child

      Correct Answer: Continue sulphasalazine and stop methotrexate

      Explanation:

      It is common for rheumatology clinics to encounter female patients with rheumatoid arthritis who are of child-bearing age. In such cases, the priority is to determine the need for treatment while also considering the risks of disease flares and potential harm to the fetus. During pregnancy, most RA patients experience an improvement in their condition, but caution must be exercised with certain medications. Methotrexate, for instance, is highly teratogenic and should be discontinued one to three months before pregnancy. On the other hand, DMARDs like hydroxychloroquine and sulphasalazine are generally considered safe to continue during pregnancy, although the latter should be avoided by male patients attempting to conceive due to the risk of oligospermia. Glucocorticoids can also be used during pregnancy, but only in low doses after 14 weeks, as there is an increased risk of cleft palate and gestational hypertension before this time. In cases where flares occur after discontinuing methotrexate, low doses of prednisolone may be an option.

      Rheumatoid arthritis (RA) management has been transformed by the introduction of disease-modifying therapies in recent years. Patients with joint inflammation should begin a combination of disease-modifying drugs (DMARD) as soon as possible. Other important treatment options include analgesia, physiotherapy, and surgery.

      In 2018, NICE updated their guidelines for RA management, recommending DMARD monotherapy with or without a short course of bridging prednisolone as the initial step. Previously, dual DMARD therapy was advocated. To monitor response to treatment, NICE suggests using a combination of CRP and disease activity (using a composite score such as DAS28).

      Flares of RA are often managed with corticosteroids, either orally or intramuscularly. Methotrexate is the most commonly used DMARD, but monitoring of FBC & LFTs is essential due to the risk of myelosuppression and liver cirrhosis. Other important side-effects include pneumonitis. Other DMARDs include sulfasalazine, leflunomide, and hydroxychloroquine.

      TNF-inhibitors are indicated for patients who have had an inadequate response to at least two DMARDs, including methotrexate. Etanercept is a recombinant human protein that acts as a decoy receptor for TNF-α and is administered subcutaneously. Infliximab is a monoclonal antibody that binds to TNF-α and prevents it from binding with TNF receptors, and is administered intravenously. Adalimumab is also a monoclonal antibody, administered subcutaneously. Risks associated with TNF-inhibitors include reactivation of tuberculosis and demyelination.

      Rituximab is an anti-CD20 monoclonal antibody that results in B-cell depletion. Two 1g intravenous infusions are given two weeks apart, but infusion reactions are common. Abatacept is a fusion protein that modulates a key signal required for activation of T lymphocytes, leading to decreased T-cell proliferation and cytokine production. It is given as an infusion but is not currently recommended by NICE.

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      • Rheumatology
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  • Question 2 - A 45-year-old man with poorly controlled diabetes and a history of social alcohol...

    Correct

    • A 45-year-old man with poorly controlled diabetes and a history of social alcohol consumption presents with an acutely swollen and red mid foot that has been bothering him for the past three weeks. He denies any recent foot injuries. Upon examination, the mid foot is warm and pedal pulses are intact. However, there is a glove and stocking distribution of sensory loss bilaterally. Recent blood tests reveal normal FBC, CRP, and UEC, and a foot radiograph appears normal. What should be the next step in managing this patient?

      Your Answer: Immobilise in a plaster cast

      Explanation:

      Charcot neuroarthropathy is a condition that presents as a warm, swollen, erythematous foot and ankle, and is often seen in patients with long-standing diabetes and peripheral neuropathy. It can progress through four stages, with radiographs being an important part of diagnosis and treatment involving immobilization and avoidance of weight-bearing. Bisphosphonates and surgery may also be used in severe cases. Antibiotics are not indicated unless there is evidence of cellulitis. The cause of peripheral neuropathy is most likely diabetes.

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      • Rheumatology
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  • Question 3 - A 49-year-old woman visits her doctor complaining of joint swelling, morning stiffness, weight...

    Incorrect

    • A 49-year-old woman visits her doctor complaining of joint swelling, morning stiffness, weight loss, myalgia, and general malaise. She is concerned that she may have hyperthyroidism because her mother and grandmother both had thyroidectomy at a young age.

      During the examination, the doctor notes a mild erythema around the patient's nose, but not in the nasolabial folds. There is no proximal myalgia, and cardiovascular and respiratory examination is normal. No goitre is present.

      Blood tests reveal an elevated ESR, prompting an autoimmune profile. The results show a positive rheumatoid factor, negative anti-CCP, ANA at 1:320, negative ANCA, positive anti-DsDNA, normal C3/C4 levels, and positive anti-RNP. The patient's TSH level is 4.5 miU/l, and T4 is 12 pmol/l.

      Based on the above information, what is the most likely diagnosis?

      Your Answer: Mixed Connective Tissue Disorder

      Correct Answer: Systemic lupus erythematosus

      Explanation:

      This patient has positive antibodies associated with rheumatological conditions, but it is important to focus on her specific symptoms and complaints. Based on her gender, age, and medical history, it is likely that she has a systemic autoimmune condition, with Systemic lupus erythematosus being the most probable diagnosis due to her examination results. The patient’s fleeting joint swelling is a common symptom of SLE, along with non-specific symptoms and the characteristic butterfly rash. While palindromic rheumatoid arthritis could explain the fleeting arthralgia, it does not account for the other observed signs. Polymyalgia rheumatica is unlikely due to the patient’s age and would not present with the same findings.

      Systemic lupus erythematosus (SLE) can be investigated through various tests, including antibody tests. ANA testing is highly sensitive and useful for ruling out SLE, but it has low specificity. About 99% of SLE patients are ANA positive. Rheumatoid factor testing is positive in 20% of SLE patients. Anti-dsDNA testing is highly specific (>99%) but less sensitive (70%). Anti-Smith testing is also highly specific (>99%) but has a lower sensitivity (30%). Other antibody tests that can be used include anti-U1 RNP, SS-A (anti-Ro), and SS-B (anti-La).

      Monitoring of SLE can be done through various markers, including inflammatory markers such as ESR. During active disease, CRP levels may be normal, and a raised CRP may indicate an underlying infection. Complement levels (C3, C4) are low during active disease due to the formation of complexes that lead to the consumption of complement. Anti-dsDNA titres can also be used for disease monitoring, but it is important to note that they are not present in all SLE patients. Overall, these investigations can help diagnose and monitor SLE, allowing for appropriate management and treatment.

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      • Rheumatology
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  • Question 4 - A 55-year-old woman presents with a history of breathlessness on exertion, leg swelling,...

    Incorrect

    • A 55-year-old woman presents with a history of breathlessness on exertion, leg swelling, and a recent rash on her face. She was diagnosed with congestive heart failure and acute kidney injury and is currently being treated with diuretics. An echocardiogram revealed severe left ventricular dysfunction. During her hospitalization, she experienced a ventricular fibrillation cardiac arrest, which was successfully treated. She is now receiving amiodarone and betablocker. Her blood tests did not show any electrolyte abnormalities, and a coronary angiogram revealed normal coronary arteries. She was subsequently diagnosed with systemic lupus erythematosus and lupus myocarditis and is receiving appropriate immunosuppressive treatment. What is the best management for her ventricular fibrillation cardiac arrest?

      Your Answer: Implantable cardioverter defibrillator immediately and stop amiodarone

      Correct Answer: Continue amiodarone and medical treatment of lupus and review progress in clinic to decide about implantable cardioverter defibrillator

      Explanation:

      The management of cardiac arrest survivors involves various aspects that need to be considered. As per the ACC/AHA/HRS 2008 guidelines, survivors of ventricular fibrillation cardiac arrest should undergo investigations to identify the cause of the arrest and exclude any reversible causes before implanting a cardioverter defibrillator (ICD). Electrolyte abnormalities are common reversible causes, but there are other causes as well.

      In a case where a patient has severe left ventricular systolic dysfunction due to lupus myocarditis, medical management is necessary before considering ICD insertion. The underlying condition needs to be treated, and the patient’s left ventricular function should be monitored with repeat echocardiograms. If the function does not improve, an ICD may be indicated in the future.

      It is important to note that amiodarone, a medication commonly used to treat cardiac arrhythmias, has several side effects. Therefore, it should only be continued if there is a justifiable reason to do so.

      Understanding Systemic Lupus Erythematosus: A Multisystem Autoimmune Disorder

      Systemic lupus erythematosus (SLE) is a complex autoimmune disorder that affects multiple systems in the body. It typically develops in early adulthood and is more common in women and individuals of Afro-Caribbean descent. The condition is characterized by a range of symptoms, including fatigue, fever, mouth ulcers, and lymphadenopathy.

      SLE can also affect the skin, causing a malar (butterfly) rash that spares the nasolabial folds, as well as a discoid rash that is scaly, erythematous, and well-demarcated in sun-exposed areas. Other skin symptoms may include photosensitivity, Raynaud’s phenomenon, livedo reticularis, and non-scarring alopecia.

      Musculoskeletal symptoms of SLE may include arthralgia and non-erosive arthritis, while cardiovascular symptoms may include pericarditis and myocarditis. Respiratory symptoms may include pleurisy and fibrosing alveolitis, and renal symptoms may include proteinuria and glomerulonephritis, with diffuse proliferative glomerulonephritis being the most common type.

      Finally, neuropsychiatric symptoms of SLE may include anxiety and depression, psychosis, and seizures. Overall, SLE is a complex and challenging condition that requires careful management and ongoing support.

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      • Rheumatology
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  • Question 5 - A 45-year-old woman presents to an outpatient rheumatology clinic with a history of...

    Correct

    • A 45-year-old woman presents to an outpatient rheumatology clinic with a history of Systemic lupus erythematosus for the past 5 years. She initially presented with fatigue, anaemia and a rash. Her medical history includes hypertension, gout and psoriasis. Despite noticing deformities in her hand joints, she denies any functional impairment or pain.

      Upon examination, the patient exhibits marked reducible ulnar subluxation and deviation at the MCP joints, which is symmetrical. X-rays of her hands reveal no erosions.

      What is the most likely diagnosis?

      Your Answer: Jaccoud's arthropathy

      Explanation:

      The correct answer is Jaccoud’s arthropathy, which is non-erosive and associated with systemic lupus erythematosus. This is because the absence of pain is a key factor, which is likely to be present in options B to E. The presence of psoriasis is a distracting factor, and gout typically affects the first metatarsal head. Sarcoid arthropathy is also very rare.

      Understanding Systemic Lupus Erythematosus: A Multisystem Autoimmune Disorder

      Systemic lupus erythematosus (SLE) is a complex autoimmune disorder that affects multiple systems in the body. It typically develops in early adulthood and is more common in women and individuals of Afro-Caribbean descent. The condition is characterized by a range of symptoms, including fatigue, fever, mouth ulcers, and lymphadenopathy.

      SLE can also affect the skin, causing a malar (butterfly) rash that spares the nasolabial folds, as well as a discoid rash that is scaly, erythematous, and well-demarcated in sun-exposed areas. Other skin symptoms may include photosensitivity, Raynaud’s phenomenon, livedo reticularis, and non-scarring alopecia.

      Musculoskeletal symptoms of SLE may include arthralgia and non-erosive arthritis, while cardiovascular symptoms may include pericarditis and myocarditis. Respiratory symptoms may include pleurisy and fibrosing alveolitis, and renal symptoms may include proteinuria and glomerulonephritis, with diffuse proliferative glomerulonephritis being the most common type.

      Finally, neuropsychiatric symptoms of SLE may include anxiety and depression, psychosis, and seizures. Overall, SLE is a complex and challenging condition that requires careful management and ongoing support.

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      • Rheumatology
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  • Question 6 - A 32-year-old woman presents to the clinic with complaints of increasing fatigue over...

    Incorrect

    • A 32-year-old woman presents to the clinic with complaints of increasing fatigue over the past three months. She denies any other symptoms such as pain, bleeding, weight loss, or mood changes. Her medical history includes asthma, which has been well-controlled for over a year, and she has no known allergies. She is currently taking oral contraceptive pills. Laboratory tests reveal normal FBC, U&Es, calcium, and parathyroid hormone levels, but her vitamin D level is low at 32 nmol/L. What is the most appropriate treatment for her vitamin D deficiency?

      Your Answer: Dietary advise only

      Correct Answer: Maintenance dose vitamin D

      Explanation:

      Osteomalacia is treated by supplementing with vitamin D, often with an initial loading dose regimen. In this patient’s case, her tiredness may be explained by vitamin D insufficiency, which can be managed with a maintenance dose of vitamin D. A loading dose would only be necessary if her serum level was less than 30 nmol/L. Patients with adequate levels, above 50 nmol/L, can benefit from dietary advice. As the patient’s calcium levels are normal and there is no evidence of a low calcium diet, calcium supplementation is not necessary.

      To summarize, the treatment for vitamin D deficiency or insufficiency depends on the serum levels. Adequate levels can be maintained with dietary recommendations, while insufficient levels require a maintenance dose of vitamin D. Deficient levels may require a loading dose of vitamin D.

      Understanding Osteomalacia: Causes, Features, Investigation, and Treatment

      Osteomalacia is a condition characterized by the softening of bones due to low levels of vitamin D, which leads to a decrease in bone mineral content. While rickets is the term used for this condition in growing children, osteomalacia is the preferred term for adults. The causes of osteomalacia include vitamin D deficiency, malabsorption, lack of sunlight, diet, chronic kidney disease, drug-induced factors, inherited factors, liver disease, and coeliac disease.

      The features of osteomalacia include bone pain, bone/muscle tenderness, fractures (especially femoral neck), proximal myopathy, and a waddling gait. To investigate this condition, blood tests are conducted to check for low vitamin D levels, low calcium and phosphate levels (in around 30% of patients), and raised alkaline phosphatase (in 95-100% of patients). X-rays may also show translucent bands known as Looser’s zones or pseudofractures.

      The treatment for osteomalacia involves vitamin D supplementation, with a loading dose often needed initially. Calcium supplementation may also be necessary if dietary calcium is inadequate. By understanding the causes, features, investigation, and treatment of osteomalacia, individuals can take steps to prevent and manage this condition.

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      • Rheumatology
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  • Question 7 - A 35-year-old Hispanic woman presented with a 3-week history of fever and chills...

    Correct

    • A 35-year-old Hispanic woman presented with a 3-week history of fever and chills followed by a widespread papular rash over her body. Two weeks ago, she noticed that her elbow and knee were swollen and tender. The inflammation resolved a few days later, but now her left ankle is very swollen and tender and she can barely weight-bear on it. She works as a nurse and she was in Mexico until 3 weeks ago on a vacation.
      On examination, her left ankle is hot and swollen and there are a few vesicular lesions on her face.
      Investigations reveal the following:

      Haemoglobin (Hb) 142 g/l 135–175 g/l
      White cell count (WCC) 12.5 × 109/l 4.0–11.0 × 109/l
      Sodium (Na+) 142 mmol/l 135–145 mmol/l
      Potassium (K+) 4.2 mmol/l 3.5–5 mmol/l
      Creatinine (Cr) 92 μmol/l 50–120 μmol/l
      Rheumatoid factor (RF) Negative
      Antinuclear antibody (ANA) Absent
      Synovial fluid white cells > 15 000/μl
      What is the likely diagnosis?

      Your Answer: Gonococcal arthritis

      Explanation:

      Disseminated gonococcal infection is a sexually transmitted bacterial infection caused by Neisseria gonorrhoeae. This patient presents with the dermatitis–polyarthritis–tenosynovitis syndrome, which includes symptoms such as fever, symmetrical polyarthralgia, tenosynovitis, and dermatitis. Diagnosis is made through culture of synovial fluid, skin lesions, and urethral swabs. Treatment involves NSAIDs for symptomatic relief, as well as ceftriaxone and azithromycin. Acute gout, reactive arthritis, tuberculous arthritis, and pseudo-gout were considered in the differential diagnosis but were ruled out based on the patient’s age, social history, and associated signs. This case highlights the importance of considering disseminated gonococcal infection in patients presenting with joint symptoms and a history of sexual activity.

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      • Rheumatology
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  • Question 8 - A 68-year-old Caucasian patient with Granulomatosis with polyangiitis has been receiving monthly i.v....

    Correct

    • A 68-year-old Caucasian patient with Granulomatosis with polyangiitis has been receiving monthly i.v. cyclophosphamide and oral prednisolone for six months, resulting in well-controlled vasculitis. The next step is to start him on azathioprine. All his blood tests, including full blood count, urea electrolytes, creatinine, and liver function tests, are normal. What screening test should be done before initiating azathioprine therapy?

      Your Answer: Blood for thiopurine methyltransferase

      Explanation:

      Importance of Checking TPMT Levels Before Starting Azathioprine

      Prior to initiating treatment with azathioprine, it is crucial to check the patient’s blood for thiopurine methyltransferase (TPMT) levels. Azathioprine is metabolized into 6-mercaptopurine (6-MP) in the body, which can either be converted into inactive 6-methyl mercaptopurine or methylated into active compounds by TPMT. In Caucasians, 89% of individuals have normal or high levels of TPMT, while 11% have low levels and 0.3% are deficient in TPMT. Administering azathioprine to those who are TPMT deficiency can lead to severe side effects such as myelosuppression.

      Patients with normal or high levels of TPMT can safely begin treatment with azathioprine. However, those with low levels of TPMT should be closely monitored and given low doses of azathioprine under expert supervision. Therefore, it is essential to check TPMT levels before starting azathioprine to ensure the safety and efficacy of the treatment.

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      • Rheumatology
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  • Question 9 - A 35-year-old woman visits the Rheumatology Clinic for evaluation. She reports experiencing pain...

    Correct

    • A 35-year-old woman visits the Rheumatology Clinic for evaluation. She reports experiencing pain in multiple joints, particularly in her back, wrists, ankles, and feet, with noticeable involvement of the distal interphalangeal joints. During the examination, psoriatic patches are observed on her elbows, and her nails show onycholysis, transverse ridging, and pitting. There is also swelling, pain, and limited movement in her DIP. Despite being prescribed methotrexate, there is no improvement in her condition. You decide to treat her with adalimumab. What is the mechanism of action of adalimumab?

      Your Answer: TNF-α

      Explanation:

      Biologic Agents for Psoriasis and Psoriatic Arthritis

      Psoriasis and psoriatic arthritis are chronic inflammatory conditions that can significantly impact a patient’s quality of life. Biologic agents have revolutionized the treatment of these conditions, offering targeted therapy with fewer side effects than traditional systemic treatments.

      Adalimumab, etanercept, and infliximab are anti-TNF agents that have shown efficacy as second-line agents in the management of psoriasis and psoriatic arthritis. These drugs target TNF-α, a cytokine that plays an important role in joint inflammation and destruction.

      Ustekinumab is an IL-12/23 antagonist that has recently been approved for the treatment of psoriasis and psoriatic arthritis. This drug targets IL-12 and IL-23, two cytokines that are involved in the inflammatory response.

      While erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may fall in response to anti-TNF therapy, they are more non-specific markers of inflammation. Therefore, they are not reliable indicators of treatment response.

      In conclusion, biologic agents offer a promising treatment option for patients with psoriasis and psoriatic arthritis. By targeting specific cytokines involved in the inflammatory response, these drugs can provide effective relief with fewer side effects than traditional systemic treatments.

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      • Rheumatology
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  • Question 10 - A 67-year-old woman presents to the oncology clinic for follow-up. She has advanced...

    Correct

    • A 67-year-old woman presents to the oncology clinic for follow-up. She has advanced breast cancer that is estrogen receptor positive and HER2 negative, with metastasis to her ribs, thoracic vertebrae, and right humerus. She previously underwent a right mastectomy and received first-line chemotherapy, but has declined further chemotherapy.

      The patient has been experiencing back and rib pain, which was relieved by external beam radiotherapy. She was prescribed alendronate to prevent pathological fractures, but has been suffering from nausea, severe acid reflux, and epigastric discomfort that was not alleviated by a proton pump inhibitor. Alendronate was discontinued, and risedronate was tried, but it had similar effects and was also stopped.

      What is the most appropriate medication to prescribe to prevent skeletal-related events in this patient?

      Your Answer: Denosumab

      Explanation:

      Due to oesophageal irritation and nausea, bisphosphonates are not a viable option for this patient with bone metastases who is at risk of pathological fracture. Instead, denosumab, a monoclonal antibody that inhibits RANKL on osteoclasts, is recommended by NICE for women who have been intolerant to bisphosphonates and have osteoporosis or advanced breast cancer with bone metastases. Strontium ranelate is only recommended for primary prevention of osteoporotic fractures in at-risk women who cannot tolerate bisphosphonates, and lapatinib and letrozole are used to treat HER2 positive breast cancer and oestrogen receptor positive breast cancer, respectively, but have not been shown to reduce skeletal related events in patients with these cancer types.

      Denosumab for Osteoporosis: Uses, Side Effects, and Safety Concerns

      Denosumab is a human monoclonal antibody that inhibits the development of osteoclasts, the cells that break down bone tissue. It is given as a subcutaneous injection every six months to treat osteoporosis. For patients with bone metastases from solid tumors, a larger dose of 120mg may be given every four weeks to prevent skeletal-related events. While oral bisphosphonates are still the first-line treatment for osteoporosis, denosumab may be used as a next-line drug if certain criteria are met.

      The most common side effects of denosumab are dyspnea and diarrhea, occurring in about 1 in 10 patients. Other less common side effects include hypocalcemia and upper respiratory tract infections. However, doctors should be aware of the potential for atypical femoral fractures in patients taking denosumab and should monitor for unusual thigh, hip, or groin pain.

      Overall, denosumab is generally well-tolerated and may have an increasing role in the management of osteoporosis, particularly in light of recent safety concerns regarding other next-line drugs. However, as with any medication, doctors should carefully consider the risks and benefits for each individual patient.

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      • Rheumatology
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SESSION STATS - PERFORMANCE PER SPECIALTY

Rheumatology (6/10) 60%
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