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Question 1
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A 67-year-old man presents to the hospital with acute pain in his left hip after a fall. His medical history includes Paget's disease and depression, for which he takes venlafaxine. As a child, he was treated for acute lymphocytic leukaemia. The orthopaedic team has requested advice as the CT scan shows demineralisation in the femur and pelvis. A DEXA scan confirms osteoporosis. Blood tests reveal a calcium level of 2.0 mmol/L (2.1-2.6), phosphate level of 0.9 mmol/L (0.8-1.4), magnesium level of 0.9 mmol/L (0.7-1.0), TSH level of 8.9 mU/L (0.5-5.5), free T4 level of 3 pmol/L (9.0 - 18), free testosterone level of 82 ng/dL (in ages >50 years: 193 - 740), and ALP level of 202 U/L (60-306). What factors in this man's medical history and blood tests could contribute to his development of osteoporosis?
Your Answer: Low testosterone levels
Explanation:To check for osteoporosis in a man, it is important to check his testosterone levels as low levels are associated with reduced bone mineral density and increased risk of fractures. Acute lymphocytic leukemia is not linked to osteoporosis, but multiple myeloma and lymphomas are. Hyperthyroidism, not hypothyroidism, is associated with osteoporosis. Paget’s disease is a separate condition from osteoporosis and is associated with increased osteoclast activity and abnormal new bone formation. While SSRIs and anti-epileptics are linked to osteoporosis, there is no known link between venlafaxine and the condition.
Osteoporosis is a condition that is more prevalent in women and increases with age. However, there are many other risk factors and secondary causes of osteoporosis. Some of the most significant risk factors include a history of glucocorticoid use, rheumatoid arthritis, alcohol excess, parental hip fracture history, low body mass index, and current smoking. Other risk factors include a sedentary lifestyle, premature menopause, certain ethnicities, endocrine disorders, gastrointestinal disorders, chronic kidney disease, and certain genetic disorders. Additionally, certain medications such as SSRIs, antiepileptics, and proton pump inhibitors may worsen osteoporosis.
If a patient is diagnosed with osteoporosis or has a fragility fracture, further investigations may be necessary to identify the cause of osteoporosis and assess the risk of subsequent fractures. Recommended investigations include a history and physical examination, blood tests such as a full blood count, urea and electrolytes, liver function tests, bone profile, CRP, and thyroid function tests. Other procedures may include bone densitometry, lateral radiographs, protein immunoelectrophoresis, and urinary Bence-Jones proteins. Additionally, markers of bone turnover and urinary calcium excretion may be assessed. By identifying the cause of osteoporosis and contributory factors, healthcare providers can select the most appropriate form of treatment.
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This question is part of the following fields:
- Rheumatology
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Question 2
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A 50-year-old female presents to the acute medical unit with fever and rigors. She has a medical history of rheumatoid arthritis and is currently taking methotrexate and sulfasalazine. Recently, she was treated with trimethoprim for a urinary tract infection.
Upon examination, her blood results show a hemoglobin level of 110 g/l, platelets at 94 * 109/l, and a white blood cell count of 1.2 * 109/l. Her neutrophil count is 0.6 * 109/l, lymphocyte count is 0.4 * 109/l, and her CRP level is 212 mg/l. Her sodium level is 138 mmol/l, potassium level is 3.8 mmol/l, urea level is 7.8 mmol/l, and creatinine level is 104 µmol/l.
What is the recommended treatment for this patient?Your Answer: Folinic acid
Explanation:When a patient experiences methotrexate toxicity, folinic acid is the preferred treatment. Methotrexate is a dihydrofolate reductase antagonist, and as such, taking trimethoprim, a selective inhibitor of dihydrofolate reductase, is strictly prohibited. In this case, the patient has developed bone marrow suppression and neutropenic sepsis due to methotrexate toxicity. Folinic acid can bypass the effects of methotrexate on dihydrofolate reductase and replenish the body’s supply of folate. Therefore, it is crucial that the patient receives folinic acid immediately. Platelet transfusions are typically only given when the platelet count drops below 50 * 109/l. Additionally, the patient should be treated with broad-spectrum antibiotics for neutropenic sepsis. While urinary alkalisation with sodium bicarbonate has been shown to enhance drug excretion, the evidence supporting its effectiveness is limited.
Methotrexate is an antimetabolite that hinders the activity of dihydrofolate reductase, an enzyme that is crucial for the synthesis of purines and pyrimidines. It is a significant drug that can effectively control diseases, but its side-effects can be life-threatening. Therefore, careful prescribing and close monitoring are essential. Methotrexate is commonly used to treat inflammatory arthritis, especially rheumatoid arthritis, psoriasis, and acute lymphoblastic leukaemia. However, it can cause adverse effects such as mucositis, myelosuppression, pneumonitis, pulmonary fibrosis, and liver fibrosis.
Women should avoid pregnancy for at least six months after stopping methotrexate treatment, and men using methotrexate should use effective contraception for at least six months after treatment. Prescribing methotrexate requires familiarity with guidelines relating to its use. It is taken weekly, and FBC, U&E, and LFTs need to be regularly monitored. Folic acid 5mg once weekly should be co-prescribed, taken more than 24 hours after methotrexate dose. The starting dose of methotrexate is 7.5 mg weekly, and only one strength of methotrexate tablet should be prescribed.
It is important to avoid prescribing trimethoprim or co-trimoxazole concurrently as it increases the risk of marrow aplasia. High-dose aspirin also increases the risk of methotrexate toxicity due to reduced excretion. In case of methotrexate toxicity, the treatment of choice is folinic acid. Overall, methotrexate is a potent drug that requires careful prescribing and monitoring to ensure its effectiveness and safety.
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This question is part of the following fields:
- Rheumatology
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Question 3
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A 29-year-old female presents with increasing fatigue. She also complains of joint pains affecting several joints. There is associated joint stiffness lasting about 20-30 mins. She also gets a skin rash, especially in sun-exposed areas. There is no significant past medical history and she is not using any regular medications. She works as an office clerk and the joint pains are making it difficult for her to continue her work normally. Following are the results of her investigations:
Hb 9.3 g/l Na+ 136 mmol/l Bilirubin 98 µmol/l
Platelets 99 * 109/l K+ 4.2 mmol/l ALP 89 u/l
WBC 7.3 * 109/l Urea 4.5 mmol/l ALT 23 u/l
Neuts 3.4 * 109/l Creatinine 87 µmol/l γGT 43 u/l
Lymphs 2.7 * 109/l CRP <1 mg/l Albumin 32 g/l
Eosin 0.2 * 109/l C3, C4 low
Chest x-ray and ECG were normal. Urine exam showed protein +++.
What is a characteristic feature of the underlying condition in this patient?Your Answer: Normal CRP
Explanation:It is highly unlikely for a patient to have SLE with a negative ANA, as it is a common marker for the disease.
SLE is characterized by periods of remission and relapse.
Individuals with SLE have an increased risk of developing thrombosis.
CRP levels are typically normal in SLE, unless there is a concurrent infection.
Anti-CCP is more specific to rheumatoid arthritis than SLE.
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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This question is part of the following fields:
- Rheumatology
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Question 4
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A 30-year-old woman presents to the hospital with recurrent fevers and joint pain. She denies any recent foreign travel or recreational drug use. On examination, she has a salmon-pink rash on her trunk and mild cervical lymphadenopathy. Blood results show elevated WBC count, ESR, and ferritin levels. What is the recommended initial treatment for the likely diagnosis?
Your Answer: Ibuprofen
Explanation:The first line of treatment for Still’s disease is NSAIDs, not steroids. This condition is diagnosed based on the presence of fevers, joint pain, and a salmon-colored bumpy rash, and is typically diagnosed clinically. Patients with AOSD often have high ferritin levels and elevated ESR, as well as liver function test abnormalities. NSAIDs such as ibuprofen or naproxen are the initial treatment options, followed by immunosuppressive therapy to control the disease and induce remission in severe cases. Prednisolone is a steroid that can be used for this purpose. Ciprofloxacin is an antibiotic that can treat various infections, including typhoid fever, which can also cause a salmon-pink rash. However, given the negative travel history, typhoid fever is highly unlikely. Azathioprine is an immunosuppressive medication commonly used in rheumatoid arthritis, granulomatosis with polyangiitis, and other conditions, as well as post-kidney transplant to prevent rejection.
Still’s disease in adults is a condition that has a bimodal age distribution, affecting individuals between the ages of 15-25 years and 35-46 years. The disease is characterized by symptoms such as arthralgia, elevated serum ferritin, a salmon-pink maculopapular rash, pyrexia, lymphadenopathy, and a daily pattern of worsening joint symptoms and rash in the late afternoon/early evening. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) tests are negative, making the diagnosis of Still’s disease in adults challenging. The Yamaguchi criteria is the most widely used criteria, with a sensitivity of 93.5%.
Management of Still’s disease in adults involves the use of NSAIDs as first-line treatment to manage fever, joint pain, and serositis. It is recommended to trial NSAIDs for at least a week before adding steroids. While steroids may control symptoms, they do not improve prognosis. If symptoms persist, methotrexate, IL-1, or anti-TNF therapy can be considered. Overall, the management of Still’s disease in adults requires a multidisciplinary approach and close monitoring to ensure optimal outcomes.
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This question is part of the following fields:
- Rheumatology
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Question 5
Correct
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A 67-year-old male smoker presents with progressive difficulty in walking long distances. He also has trouble lifting heavy objects.
He has 4/5 power proximally, with intact reflexes and downward going plantars.
Dermatomyositis was diagnosed based on the following blood tests, and muscle biopsy:
Hb 118 g/L (130-180)
WBC 7.2 ×109/L (4-11)
Neutrophils 80% (40-75)
Platelet 190 ×109/L (150-400)
ESR 70 mm/hr (0-20)
CK 250 IU/L (24-195)
CRP 140 mg/L (<10)
Urea, electrolytes and creatinine are normal. CXR is also normal.
Anti-nuclear antibody negative 1:12800
Anti-ENA antibody negative
What is the most appropriate next investigation?Your Answer: CT chest, abdomen pelvis
Explanation:Myositis and its Association with Malignancy
Myositis is a condition that is associated with malignancy, with dermatomyositis having a stronger association than polymyositis. Patients with polymyositis who do not have any associated antibodies, such as anti-Jo1 or anti-Pm/Scl, should be screened for malignancies that are known to be associated with myositis. These malignancies include ovary, stomach, pancreas, colon, lung, and breast in women, although this is not an exhaustive list. It is important for healthcare professionals to be aware of this association and to screen patients accordingly to ensure early detection and treatment of any underlying malignancies.
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This question is part of the following fields:
- Rheumatology
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Question 6
Incorrect
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A 46-year-old woman comes to the Rheumatology Clinic complaining of progressive swelling in both hands and shortness of breath. She has noticed her hands becoming whiter at times for many years. Lately, she has started experiencing dyspnoea on exertion of up to 400 yards, with occasional palpitations.
The patient has been smoking 15 cigarettes a day for 20 years and drinks 14 units of alcohol a week. She is currently taking lansoprazole for gastro-oesophageal reflux disease, as prescribed by her General Practitioner.
During the examination, evidence of calcinosis is found in her hands. Autoantibodies are checked, which reveal a positive anti-centromere antibody. What is the most probable cause of this patient's exertional dyspnoea?Your Answer: Chronic obstructive pulmonary disease
Correct Answer: Pulmonary artery hypertension
Explanation:Limited Cutaneous Systemic Sclerosis: A Rare Autoimmune Disease with Pulmonary Hypertension as a Significant Risk
Limited cutaneous systemic sclerosis, also known as CREST syndrome, is a rare autoimmune disease characterized by tissue fibrosis, small blood vessel vasculopathy, and autoimmunity. It is commonly associated with anti-centromere antibodies and pulmonary artery hypertension, which usually appears later in the disease process. This condition is differentiated from diffuse scleroderma, which involves both cutaneous and visceral involvement. Fibrosing interstitial lung disease is a common differential diagnosis, but it is more commonly seen in systemic sclerosis with positive anti-topoisomerase 1 autoantibodies. Chronic obstructive pulmonary disease, chronic pulmonary emboli, and congestive cardiac failure are unlikely to be associated with limited cutaneous systemic sclerosis. Therefore, in patients presenting with dyspnea on exertion, palpitations, and positive anti-centromere antibodies, limited cutaneous systemic sclerosis with pulmonary hypertension should be considered as a significant risk.
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This question is part of the following fields:
- Rheumatology
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Question 7
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A 56-year-old patient with tophaceous gout is unable to tolerate allopurinol tablets due to hypersensitivity reaction. The patient is considering long-term urate-lowering treatment with febuxostat. What is the mechanism of action of febuxostat?
Your Answer: Xanthine oxidase inhibitor
Explanation:Febuxostat is a xanthine oxidase inhibitor that works by blocking the enzyme responsible for the production of uric acid in the body. By reducing the amount of uric acid produced, febuxostat helps to lower the levels of uric acid in the blood, which can prevent the formation of urate crystals that cause gout. Unlike allopurinol, febuxostat does not require dose adjustment in patients with renal impairment and has a lower risk of hypersensitivity reactions.
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This question is part of the following fields:
- Rheumatology
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Question 8
Incorrect
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A 50-year-old man with RA is currently on s.c. adalimumab (40 mg/2week), methotrexate 15 mg/week, folic acid 5 mg/day, and prednisolone 5 mg/day. He presents to the MAU with a painful swollen right ankle and is unable to weight bear on the right leg. There are no drug allergies. On examination, his temperature is 38.8℃, BP is 90/60 mm Hg, and pulse is 110/min. The right ankle is warm, tender, and there is a tense joint effusion. Bloods including blood cultures have been collected, and fluid resuscitation has been initiated. What is the most appropriate step in his management?
Your Answer: Urgently arrange ankle aspiration
Correct Answer: Administer i.v. flucloxacillin, and arrange joint aspiration urgently
Explanation:Diagnosis of Septic Arthritis
Septic arthritis should be ruled out in patients with acute monoarthritis. Even in immunosuppressed individuals, septic arthritis can present as oligo- or polyarticular and may not always be accompanied by fever. To diagnose septic arthritis, joint aspiration is crucial, followed by microscopy and culture of the synovial fluid. However, if the patient is in septic shock, administering antibiotics takes priority over collecting synovial fluid, especially if it may delay antibiotic administration.
MRI scans may reveal non-specific changes such as effusion, synovial proliferation, and bone marrow edema, which are not unique to septic arthritis. A bone scan can show increased uptake at the inflamed joint, but it cannot differentiate between infective and other causes. Therefore, joint aspiration remains the gold standard for diagnosing septic arthritis.
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This question is part of the following fields:
- Rheumatology
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Question 9
Correct
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A 35-year-old woman with a history of rheumatoid arthritis presents with severe pains throughout her body, fatigue, and difficulty concentrating. What could be the underlying cause of her new symptoms?
Your Answer: Fibromyalgia
Explanation:The individual is experiencing chronic widespread pain, along with lethargy and difficulty concentrating. Palpation reveals multiple tender points. While immunological results align with a previous diagnosis of rheumatoid arthritis, there is no indication of a flare-up or the development of a new connective tissue disease or myositis. Chronic regional pain syndrome typically involves persistent burning pain in one limb following a minor injury, but this is not the case here. The symptoms are indicative of fibromyalgia, which is not a diagnosis of exclusion and can coexist with other conditions.
Fibromyalgia is a condition that causes widespread pain throughout the body, along with tender points at specific anatomical sites. It is more common in women and typically presents between the ages of 30 and 50. Other symptoms include lethargy, cognitive impairment (known as fibro fog), sleep disturbance, headaches, and dizziness. Diagnosis is made through clinical evaluation and the presence of tender points. Management of fibromyalgia is challenging and requires an individualized, multidisciplinary approach. Aerobic exercise is the most effective treatment, along with cognitive behavioral therapy and medication such as pregabalin, duloxetine, and amitriptyline. However, there is a lack of evidence and guidelines to guide treatment.
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This question is part of the following fields:
- Rheumatology
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Question 10
Incorrect
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A 63-year-old man presents with a week of right-sided shoulder pain. He denies any trauma and denies radiation of the pain. There is no associated weakness or numbness. The pain is more noticeable at the extremities of movement and is affecting activities of daily living. His past medical history includes type 2 diabetes mellitus and asthma.
Upon examination, the affected shoulder is painful and restricted, with both active and passive movement. External rotation is most severely limited. Pain is elicited when applying direct pressure to the coracoid process.
What is the most appropriate next step given the most likely diagnosis?Your Answer: Arthroscopy
Correct Answer: Physiotherapy
Explanation:Adhesive capsulitis, also known as frozen shoulder, can be diagnosed clinically without the need for imaging or arthroscopy. The patient’s reduced range of motion, pain, and greater impairment in external rotation, along with their history of diabetes mellitus, suggest adhesive capsulitis. Physiotherapy is the recommended treatment and should be initiated early on. Arthroscopy is not appropriate at this stage and should only be considered as a last resort. MRI scanning is unnecessary unless the diagnosis is unclear and other conditions need to be ruled out. Ultrasound may be considered before MRI if there is uncertainty in the diagnosis, but in this case, the clinical presentation is sufficient for diagnosis.
Adhesive capsulitis, also known as frozen shoulder, is a common cause of shoulder pain that is more prevalent in middle-aged women. The exact cause of this condition is not fully understood. It is associated with diabetes mellitus, with up to 20% of diabetics experiencing an episode of frozen shoulder. Symptoms typically develop over a few days and affect external rotation more than internal rotation or abduction. Both active and passive movement are affected, and patients usually experience a painful freezing phase, an adhesive phase, and a recovery phase. Bilateral frozen shoulder occurs in up to 20% of patients, and the episode typically lasts between 6 months and 2 years.
The diagnosis of frozen shoulder is usually made based on clinical presentation, although imaging may be necessary for atypical or persistent symptoms. There is no single intervention that has been proven to improve long-term outcomes. Treatment options include nonsteroidal anti-inflammatory drugs (NSAIDs), physiotherapy, oral corticosteroids, and intra-articular corticosteroids. It is important to note that the management of frozen shoulder should be tailored to the individual patient, and a multidisciplinary approach may be necessary for optimal outcomes.
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This question is part of the following fields:
- Rheumatology
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