Specialist & Consultant Rheumatology oral assessment prep

Knowledge: This is giant cell arteritis until proven otherwise, with cranial symptoms, jaw claudication, high inflammatory markers and a transient visual disturbance. Transient visual loss is a warning of anterior ischaemic optic neuropathy, which can cause permanent blindness within days and may affect the second eye. Treatment must start immediately and must not wait for a biopsy or ultrasound. Temporal artery ultrasound in a fast-track clinic, or biopsy, remains valid for at least a few days after glucocorticoids start. Tocilizumab reduces relapse and glucocorticoid exposure, which matters given her diabetes.

Management: I would start treatment today. Because she has visual symptoms, I would give intravenous methylprednisolone, for example 500 mg to 1 g daily for 3 days, then oral prednisolone 40 to 60 mg daily. I would arrange same-week ophthalmology review and a temporal and axillary artery ultrasound in the fast-track pathway, or a temporal artery biopsy if ultrasound is unavailable. I would monitor her glucose closely and adjust her diabetes treatment with her diabetes team. I would start bone protection, with calcium, vitamin D and a bisphosphonate after checking renal function and dental health. I would discuss tocilizumab early because of her diabetes. I would taper prednisolone gradually, guided by symptoms and markers. I would arrange imaging of the aorta at baseline or follow-up to detect large-vessel involvement.

Ethics and communication: I would explain the risk to her sight clearly, and why treatment cannot wait, so she can make an informed choice. I would discuss the side effects of long-term glucocorticoids and tocilizumab, including infection risk. She should not drive if she develops further visual symptoms, and I would document this advice.

Reference: EULAR Recommendations for the Management of Large Vessel Vasculitis: 2023 Update; BSR Guideline on Diagnosis and Treatment of Giant Cell Arteritis, 2020

Knowledge: This is scleroderma renal crisis, with malignant hypertension, acute kidney injury and microangiopathic haemolytic anaemia. Risk factors include early diffuse disease, anti-RNA polymerase III antibodies and glucocorticoid doses above about 15 mg prednisolone daily. Thrombotic thrombocytopenic purpura and other thrombotic microangiopathies are differentials, so I would check ADAMTS13 activity. ACE inhibitors transformed the outcome of this condition and are recommended immediately by EULAR. Even with treatment, many patients need dialysis, and some recover enough to stop it over months. Calcium channel blockers can be added for blood pressure control, but ACE inhibitors remain essential.

Management: I would admit her to a high-dependency unit with close blood pressure monitoring. I would start captopril, beginning at a low dose such as 6.25 to 12.5 mg and increasing every few hours, aiming to reduce blood pressure gradually over 72 hours. I would avoid rapid falls that could worsen kidney perfusion. I would add a calcium channel blocker if needed, and use an intravenous agent only for hypertensive emergency with organ damage. I would taper prednisolone to the lowest dose possible. I would monitor creatinine, haemoglobin, platelets and urine output daily. I would involve nephrology early and plan dialysis if needed. I would continue ACE inhibition even if she needs dialysis, because kidney recovery is possible.

Ethics and communication: I would explain the serious nature of the condition and the possible need for dialysis honestly. I would inform the clinic that prescribed prednisolone, in a constructive way, as a learning event. I would document the discussion and the plan. If she needs long-term dialysis, I would support her with information and counselling.

Reference: EULAR Recommendations for the Treatment of Systemic Sclerosis: 2023 Update; Firestein and Kelley's Textbook of Rheumatology

Knowledge: This is severe methotrexate toxicity caused by daily rather than weekly dosing, with mucositis, neutropenic fever and thrombocytopenia. Her kidney impairment reduces methotrexate clearance and worsens toxicity. Neutropenic sepsis is the main immediate threat to life. Folinic acid bypasses the blocked folate pathway and is the specific antidote. Glucarpidase is mainly used after high-dose intravenous methotrexate with kidney failure, not usually after oral overdose. Toxicity usually appears several days into daily dosing, and the blood count may keep falling for days after the drug is stopped. Mucositis is often the first sign and should prompt an urgent blood count. Daily dosing errors are a recognised safety risk with methotrexate.

Management: I would stop methotrexate immediately and treat her as neutropenic sepsis, taking cultures and starting broad-spectrum antibiotics within an hour. I would start intravenous folinic acid promptly, guided by local protocol and methotrexate levels, and continue until her blood count recovers. I would give intravenous fluids to support kidney function. I would discuss granulocyte colony-stimulating factor with haematology and give platelet transfusion if she bleeds. I would treat mucositis with mouth care and pain relief. I would monitor her blood count, kidney and liver function daily. I would review all her medications, including drugs that interact with methotrexate. I would report the incident through the hospital system and contact the pharmacy.

Ethics and communication: I would explain the error honestly to her and her family, with an apology. I would report it to the pharmacy and through the incident system. I would avoid blaming individuals and focus on how the error happened, including labelling, counselling at dispensing and whether she had a patient-held monitoring record. If she was not told clearly that the dose was weekly, that is a system gap the review must address. I would document the error, treatment and disclosure.

Reference: BSR and BHPR Guideline for the Prescription and Monitoring of Non-Biologic Disease-Modifying Anti-Rheumatic Drugs, 2017; Federal Decree-Law No. 4 of 2016 on Medical Liability

Knowledge: Mycophenolate is teratogenic, with risks of miscarriage and congenital malformations, so it must be stopped. Azathioprine is a safer alternative for maintaining remission in pregnancy. Hydroxychloroquine should be continued, because stopping it increases the risk of flare and it reduces the risk of congenital heart block in anti-Ro positive women. Anti-Ro antibodies can cross the placenta and cause congenital heart block in a small percentage of pregnancies. Low-dose aspirin reduces pre-eclampsia risk, especially with a history of lupus nephritis. Pregnancy outcomes are best when lupus is quiet for at least 6 months before conception.

Management: I would stop mycophenolate immediately and start azathioprine, after checking TPMT activity if available, and monitor her blood count and liver tests. I would continue hydroxychloroquine and start low-dose aspirin by 12 weeks. I would check her blood pressure, urine protein, complement and anti-dsDNA to assess lupus activity. I would arrange early review by maternal-fetal medicine and a detailed fetal anomaly scan. I would arrange serial fetal echocardiography from about 16 to 26 weeks to detect heart block, with more intensive surveillance if a previous child was affected. I would see her regularly in a joint clinic with monitoring of blood pressure, urine protein and complement, because a nephritis flare can mimic pre-eclampsia later in pregnancy. After delivery, I would discuss breastfeeding-compatible medications and contraception.

Ethics and communication: I would explain the risks from mycophenolate exposure honestly, without blame, and offer genetic counselling. Decisions about the pregnancy belong to her, and I would support her choice to continue it. I would document the counselling and the medication changes. I would also review why contraception counselling did not prevent this, as a learning point.

Reference: ACR Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases, 2020; EULAR Recommendations for Women's Health and the Management of Family Planning, Assisted Reproduction, Pregnancy and Menopause in Patients with SLE and/or APS, 2017

Knowledge: Pulmonary artery aneurysm is the most serious vascular complication of Behcet's disease and carries a high mortality from rupture. Young men with venous thrombosis are at higher risk. Venous thrombosis in Behcet's is driven by vessel wall inflammation, so immunosuppression is the main treatment. Anticoagulation in the presence of a pulmonary artery aneurysm can cause fatal haemorrhage. EULAR recommends high-dose glucocorticoids and cyclophosphamide for pulmonary artery aneurysm, with monoclonal anti-TNF antibodies for refractory disease. Embolisation is preferred over surgery for major or recurrent bleeding, because surgery carries a high mortality in this setting. Behcet's disease is relatively common along the old Silk Road, including parts of the Middle East, so the diagnosis should be familiar to GCC physicians.

Management: I would stop the plan for anticoagulation immediately. I would admit him to a high-dependency unit with close monitoring and blood available. I would start intravenous methylprednisolone and plan cyclophosphamide, or infliximab if cyclophosphamide is unsuitable, after screening for infection and tuberculosis. I would involve interventional radiology early for possible embolisation if bleeding continues. I would involve thoracic surgery as a backup. I would control cough, avoid any procedure that raises pulmonary pressure, and keep him nil by mouth until the bleeding risk is clear. I would screen for hepatitis B and tuberculosis before biological therapy. I would monitor his haemoglobin and oxygen saturation, and repeat imaging to assess the aneurysm response. Later, I would plan maintenance immunosuppression and long-term follow-up.

Ethics and communication: I would explain the risk of bleeding and the reason for stopping anticoagulation clearly. I would discuss the risks of immunosuppression, including infertility with cyclophosphamide, and offer sperm banking. I would explore why he stopped warfarin without judgement, which in hindsight may have protected him. I would document the discussions and consent.

Reference: 2018 Update of the EULAR Recommendations for the Management of Behcet's Syndrome; Firestein and Kelley's Textbook of Rheumatology

Knowledge: He has refractory rheumatoid arthritis, with high disease activity after two TNF inhibitors. The ORAL Surveillance trial showed higher rates of major cardiovascular events and cancer with tofacitinib than TNF inhibitors in older patients with cardiovascular risk factors. The European Medicines Agency now advises that JAK inhibitors should be used in people over 65, current or past long-time smokers and those with cardiovascular or cancer risk only if no suitable alternatives exist. Abatacept, rituximab and IL-6 inhibitors are appropriate alternatives for him. Smoking also reduces his response to most treatments. I would confirm adherence to methotrexate and that both TNF inhibitors were given at adequate doses before labelling him refractory. Seropositive disease predicts a good response to rituximab.

Management: I would assess his disease activity, comorbidities and cardiovascular risk. I would explain the cardiovascular and cancer risks with tofacitinib in plain numbers and compare them with the alternatives. I would write to the insurer, explaining why abatacept or rituximab is more appropriate, with references to guidance. I would offer smoking cessation support and bridge with a short course of low-dose glucocorticoid or an intra-articular injection while the appeal is decided. If he still chooses tofacitinib after an informed discussion, and no alternative is available, I would document his informed consent and monitor closely. I would review his cardiovascular risk with cardiology. I would reassess him regularly.

Ethics and communication: He is an adult with capacity, and his choice must be informed. I would explain the risks clearly and in writing. I would advocate for him with the insurer, as my duty is to his welfare, not to the cheapest option. If the insurer still refuses, I would explain the appeal routes available to him. I would document the discussion carefully.

Reference: EULAR Recommendations for the Management of Rheumatoid Arthritis with Synthetic and Biological Disease-Modifying Antirheumatic Drugs: 2022 Update; Federal Decree-Law No. 4 of 2016 on Medical Liability