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The patient in the scenario has a clinical presentation consistent with scleroderma renal crisis (SRC), which can occur in patients with diffuse cutaneous systemic sclerosis. SRC is characterized by accelerated hypertension, renal failure, microangiopathic hemolytic anemia, and in some cases, hypertensive encephalopathy. The most appropriate treatment in this scenario would be an angiotensin-converting enzyme inhibitor (ACE inhibitor), specifically E. Ramipril. Let's go through the options: Correct Answer: E. Ramipril: Ramipril, an ACE inhibitor, is the first-line treatment for scleroderma renal crisis. ACE inhibitors work by reducing angiotensin II levels, which leads to vasodilation and reduced blood pressure. They are particularly beneficial in scleroderma renal crisis because they preferentially dilate the efferent arterioles in the kidney, reducing glomerular pressure and improving renal perfusion. ACE inhibitors are the cornerstone of treatment in this setting as they can potentially halt the progression of renal failure and control the severe hypertension characteristic of this crisis. According to the latest UK guidelines, ACE inhibitors should be initiated immediately upon recognition of SRC, regardless of renal function, as they can prevent further deterioration in renal function and are lifesaving. Incorrect Answers: A. Bendroflumethiazide: Bendroflumethiazide is a thiazide diuretic commonly used to treat hypertension. However, it is not the drug of choice in scleroderma renal crisis. Diuretics like thiazides have a more limited effect on the renin-angiotensin system, which is crucial in controlling blood pressure in SRC. Additionally, thiazide diuretics are less effective when renal function is severely compromised, as seen in this patient with a creatinine level of 448 µmol/L. Using diuretics in this setting could lead to worsening renal failure without addressing the underlying cause of hypertension. B. Doxazosin: Doxazosin is an alpha-1 adrenergic receptor blocker used to lower blood pressure by relaxing blood vessels. While it may reduce blood pressure, it does not address the underlying pathophysiology of scleroderma renal crisis, which involves the renin-angiotensin system. Additionally, alpha blockers are typically used in combination with other antihypertensive agents and are not first-line treatment in SRC. Their effect on renal perfusion is also limited compared to ACE inhibitors, making them less effective in this situation. C. Indapamide: Indapamide is another thiazide-like diuretic used to manage hypertension, particularly in elderly patients. Similar to bendroflumethiazide, it does not address the renin-angiotensin system, which plays a critical role in the pathophysiology of scleroderma renal crisis. It would not be appropriate to use indapamide in this patient, especially given her severely compromised renal function. Diuretics may lead to further volume depletion and exacerbate renal injury in this scenario. D. Metoprolol: Metoprolol is a beta-blocker used to control blood pressure by reducing heart rate and the force of contraction. While it can be effective in managing hypertension, it does not directly target the renin-angiotensin system, which is dysregulated in scleroderma renal crisis. Furthermore, beta-blockers can sometimes blunt the reflex tachycardia that occurs when ACE inhibitors are started, potentially worsening renal perfusion in this context. Thus, metoprolol is not the preferred agent in this case. Summary of Why Ramipril is the Best Option: The hallmark of scleroderma renal crisis is the activation of the renin-angiotensin-aldosterone system (RAAS), leading to severe hypertension and renal failure. ACE inhibitors like ramipril block the conversion of angiotensin I to angiotensin II, leading to vasodilation, decreased aldosterone levels, and improved renal perfusion. This makes ACE inhibitors the most appropriate and effective treatment for this condition. Additionally, while starting an ACE inhibitor may initially worsen renal function (as the creatinine level may rise), continuing the treatment can improve long-term renal outcomes. This patient’s blood pressure of 204/120 mmHg, renal dysfunction (creatinine increased from 104 µmol/L to 448 µmol/L), and microangiopathic hemolytic anemia (evidenced by anemia and reticulocytosis) are all indicative of SRC, making ACE inhibitors the treatment of choice.

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Glad it helped

Please go through the following Oxford Academic article regarding Scleroderma Renal Crisis: academic.oup.com/mr/article/33/1/12/6555653

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Yes Doctor! The concepts tested remain the same for both MRCP(Ireland) & UK! The exam format albeit is different.

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The explanation is provided right after the question, which is based on haemodynamic stability. Thanks & Regards

Thanks for the comment! MRCP(UK) is considered the gold standard exam as an international benchmark for an internal medicine doctor, throughout the world. Isn't that high stakes?

Same question I have

Any update?