SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Hypertension

You are evaluating a 39-year-old man with resistant hyper-tension and severe obstructive sleep apnea (OSA). He is taking four medications including chlorthalidone, amlodipine, losartan, and spironolactone. His BP remains at 149/89 mmHg. He has marked diﬃculty wearing a CPAP mask. He has seen an article online and asks if renal denervation might help his hypertension or sleep apnea..
Question: Which of the following is correct regarding renal denervation, hypertension, and sleep apnea, based on current data?
1. Because hypertension is the result of OSA, there may be a beneﬁt of renal denervation on hypertension, but there is no reason to suggest that renal denervation would aﬀect OSA itself
2. The studies on renal denervation excluded patients with OSA, so a beneﬁt of denervation on OSA patients cannot be predicted
3. Renal denervation may decrease hypertension and the se-verity of OSA
4. Improved compliance with CPAP would have a greater eﬀect on his BP than renal denervation
Answer: C
Subject: Hypertension

You are giving a talk on diet and hypertension at a local chapter of the National Kidney Foundation. A member of the audience says, “I read on the internet that all kidney patients should eat as little potassium as possible..
Question: Does potassium have any eﬀect on BP?”
Which of the following is likely to be true regarding potassium intake and BP?
1. No correlations have been observed between potassium in-take and CKD progression
2. Hypertension correlates better with the dietary sodium/potassium ratio than with sodium intake alone
3. Potassium restriction increases natriuresis in animal studies, especially in CKD
4. Potassium restriction increases sodium delivery to the distal tubule
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 36-year-old woman presents with a 2-week history of increasing lower limb edema and weight gain. On examination, her BP is 126/88 mmHg, her weight is 67 kg (from usual weight of 58 kg), and she has periorbital, upper limb, and lower limb edema. Spot urine protein/creatinine ratio is 12.4 g/g. Her creatinine is 0.8 mg/dL, and albumin 2.2 g/dL. She undergoes kidney biopsy, and the specimen shows a normal appearance on light microscopy but diﬀuse foot process eﬀacement on electron microscopy..
Question: Regarding the management of minimal change disease, which of the following is correct?
1. When compared with corticosteroids as ﬁrst-line therapy, treatment with tacrolimus is associated with similar rates of remission and relapse
2. Alternate-day corticosteroid therapy is associated with reduced rates of remission compared with daily corticosteroid dosing
3. Rituximab has been shown to be beneﬁcial in children who fail to respond to steroid or calcineurin inhibitor therapy
4. When compared with a tacrolimus-based treatment protocol, treatment with rituximab was associated with a greater cumulative steroid dose
Answer: A
Subject: Primary and Secondary Glomerular Diseases

Focal and segmental glomerulosclerosis (FSGS) is a histologic pattern of injury that is due to several diﬀerent pathophysiologic processes. Renal biopsy is required for diagnosis of FSGS, and particular features on the histologic and ultra-structural examinations may diﬀerentiate between the causes and predict prognosis..
Question: With regard to the pathophysiology of FSGS, which of the following is correct?
1. The variant of FSGS lesion (as described in the Columbia classiﬁcation) seen on light microscopy is helpful in differentiating between primary and genetic forms of FSGS
2. Abnormalities in genes encoding glomerular basement membrane (GBM) collagen are associated with primary FSGS
3. In secondary FSGS, glomerular hypertension causes podocyte injury, leading to podocyte detachment and loss
4. The degree of foot process eﬀacement seen on electron microscopy is used to diﬀerentiate between primary and genetic FSGS
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 48-year-old man is referred to you for a second opinion. He received a diagnosis of FSGS 6 months earlier, having presented with nephrotic syndrome. His renal biopsy specimen shows FSGS, with 15% interstitial ﬁbrosis and tubular atrophy, and electron microscopy showed diﬀuse foot process eﬀacement. He was initially treated with prednisone 1 mg/kg, but tacrolimus was added as a steroid-sparing agent after 2 months because of new-onset diabetes mellitus. His proteinuria has improved from 12 g/24 hours at the time of diagnosis to 6 g/24 hours currently. His serum creatinine is 1.8 mg/dL. He wishes to discuss his treatment options with you..
Question: With regard to the treatment of FSGS, which of the following is correct?
1. Randomized controlled trials have demonstrated the eﬃcacy of rituximab in the treatment of FSGS
2. Up to 60% of patients will experience a relapse after initial treatment of FSGS with corticosteroids
3. In retrospective studies, up to 30% of adults with steroid-resistant FSGS had a potentially causative genetic mutation identiﬁed
4. Recent studies have shown beneﬁt for sodium-glucose transporter-2 (SGLT2) inhibitors in management of secondary FSGS
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 76-year-old man presents with recent-onset nephrotic syn-drome. He has 5 g of proteinuria/24 hours, his albumin is 2.8 g/dL, and creatinine is 1.5 mg/dL. He underwent renal biopsy, which was consistent with membranous nephropathy (MN), and showed negative staining for phospholipase A2 receptor (PLA2R) in the glomerular capillary walls. Anti-PLA2R serology is also negative. He undergoes a thorough evaluation for underlying malignancy, which gives negative results. He wishes to be evaluated for other causes of MN..
Question: Which of the following is correct with regard to PLA2R-negative MN?
1. Staining for exostosin 1/exostosin 2 (EXT1/EXT2) on renal biopsy specimens is associated with underlying malignancy
2. Neural epidermal growth factor like 1 protein (NELL-1)–associated MN is a rare cause of primary MN
3. Thrombospondin type 1 domain containing 7A (THSD7A)–associated MN is associated with high rates of underlying malignancy
4. Semaphorin 3B is a common cause of PLA2R-negative MN in the elderly
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 56-year-old woman is referred from her primary care provider for assessment. She recently experienced weight gain and ankle edema. She was found to have 3+ proteinuria and increased creatinine to 2.4 mg/dL, from a prior baseline of 0.8 mg/dL. Her 24-hour urine collection shows 10 g proteinuria, and her PLA2R antibody is positive at 150 RU/mL..
Question: When considering a treatment plan for this patient, which of the following is correct?
1. Renal biopsy could be deferred in this patient
2. Monitor serologies monthly and treat if PLA2R fails to decrease by > 25% in 3 months
3. In the MENTOR study, patients treated with rituximab and cyclosporine had similar estimated GFR (eGFR) at 24-month follow-up
4. In the MENTOR study, those treated with rituximab had higher rates of remission at 24-month-follow up
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 28-year-old patient is found to have dipstick hematuria during a routine physical. He is referred to nephrology for review, and his evaluation reveals BP of 148/92 mmHg, urinalysis shows 2+ heme and 1+ protein. Creatinine is 1.4 mg/dL, and his urine protein/creatinine ratio is 1680 mg/g. He undergoes renal biopsy, which shows a membranoproliferative pattern of injury, with evidence of diﬀuse proliferative glomerulonephritis. On immunoﬂuorescence, C3 staining is dominant, and electron microscopy shows electron-dense deposits in the subepithelial and subendothelial space..
Question: Which of the following is correct regarding this patients’ glomerular disease?
1. On immunoﬂuorescence, costaining with IgG and C3 is seen in dense deposit disease
2. C3 glomerulonephritis commonly presents with crescentic glomerulonephritis
3. Abnormalities of genes encoding the complement system are the most common cause of C3 glomerulopathy
4. C3 nephritic factors are seen in many patients with monoclonal gammopathy–associated C3 glomerulopathy
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 48-year-old woman comes to you for a second opinion about management of her recently diagnosed dense deposit disease. She has been prescribed lisinopril for proteinuria, but after 6 months of follow-up, she continues to have 2.5 g proteinuria/24 hours, and her creatinine has increased from 1.2 mg/dL to 1.5 mg/dL. She also has a history of prediabetes and is very reluctant to take steroids unless there is a strong indication to do so..
Question: Regarding treatment of C3 glomerulopathy (C3G), which of the following is correct?
1. Complement blockade with eculizumab has shown beneﬁt in the majority of patients with C3G
2. Treatment with mycophenolate mofetil and steroids has been associated with improved rates of remission of C3G in observational studies
3. In patients with monoclonal gammopathy–associated C3 glomerulopathy, treatment with immunosuppression shows similar outcomes to clone-based therapy
4. Use of angiotensin-converting enzyme inhibitors may be associated with reduced levels of complement activation in C3G by blocking cleavage of C3
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 38-year-old woman is referred for listing for renal trans-plantation. She experienced severe pre-eclampsia during her ﬁrst pregnancy 4 years ago, with AKI that required dialysis at the time of delivery. She was left with CKD stage IV, and despite control of her hypertension, she has progressive CKD with eGFR 20 mL/min per 1.73 m2. She has never had a renal biopsy or speciﬁc treatment for her kidney disease..
Question: Which of the following is correct regarding complement-mediated thrombotic microangiopathy (TMA)?
1. Renal cortical necrosis can be an underappreciated presentation of pregnancy-associated TMA
2. Pregnancy-associated TMA is rarely associated with abnormalities in genes encoding complement or complement regulatory proteins
3. All subtypes of complement-mediated TMA have high recurrence rates after transplantation
4. Eculizumab can be safely stopped in transplant recipients who have stable renal function at 6 months after transplantation
Answer: A
Subject: Primary and Secondary Glomerular Diseases

A 47-year-old man recently underwent renal biopsy for workup of microscopic hematuria and proteinuria, with urinary protein excretion of 1.5 g daily. His biopsy specimen showed mesangial and endocapillary proliferation, with 20% tubular atrophy/interstitial ﬁbrosis, and IgA deposition on immunoﬂuorescence. He wishes to discuss the risks and beneﬁts of immunosuppression for treatment of his IgA nephropathy..
Question: Which of the following is correct regarding treatment of IgA nephropathy?
1. In a randomized controlled trial, immunosuppression comprising cyclophosphamide and steroids, followed by azathioprine and steroids, delayed progression of CKD
2. Treatment with budesonide led to reduced proteinuria and improved eGFR in patients with patients with IgA nephropathy
3. Treatment with steroids for IgA nephropathy was well tolerated and associated with lower rates of progression of CKD
4. IgA nephropathy commonly recurs after renal transplantation
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 63-year-old man presents with a 6-week history of systemic illness including malaise, weight loss, low-grade fever, and upper respiratory symptoms. He is found to have AKI with creatinine 4.5 mg/dL and new-onset hematuria and proteinuria. ANCA is positive, with elevated anti-proteinase 3 (PR3) titer. Renal biopsy shows pauci-immune crescentic glomerulonephritis, with 20%glomerulosclerosis. He has been prescribed pulse corticosteroids and wishes to discuss his treatment options for additional therapy..
Question: Which of the following statements is correct regarding in-duction therapy for AAV?
1. The MYCYC trial showed that induction therapy with mycophenolate and steroids was inferior to cyclophosphamide and steroids for remission induction at 6-month follow-up
2. The PEXIVAS trial showed that in cases of moderate to severe renal impairment, plasma exchange was associated with reduced rates of ESKD
3. In the PEXIVAS trial, reduced corticosteroid dosing was associated with no diﬀerence in risk of ESKD, but with reduced rates of infectious complications
4. The addition of a small molecule C5a inhibitor, avacopan, to induction therapy was associated with higher rates of infectious complications as compared with standard treatment with corticosteroids and rituximab/cyclophosphamide
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 37-year-old woman received a diagnosis of AAV 6 months ago and was treated with rituximab and prednisone for induction therapy. Her ANCA titer became negative within 3 months of initiating therapy, and her peripheral B cell count was zero at most recent check. She has been on tapering dose steroids but remains on prednisone 5 mg daily. She has been reading about the risks of immunosuppression and wants to discuss the beneﬁts of aza-thioprine versus rituximab..
Question: Regarding remission maintenance in AAV, which of the following is correct?
1. In a clinical trial comparing it with maintenance therapy with azathioprine, rituximab was superior at maintaining remission of AAV
2. A ritixumab dosing strategy based on repopulation of peripheral B cells and/or changes in ANCA titer led to fewer relapses and longer relapse-free survival than regular scheduled 6-monthly dosing of rituximab
3. In a study examining the duration of treatment with oral immunosuppressants to maintain remission, there were no diﬀerences in rates of ESKD associated with the use of azathioprine and prednisone for 24 versus 48 months
4. Recent observational studies suggest that elderly patients tolerate corticosteroids well, but the use of cyclophosphamide for induction therapy is associated with higher rates of infectious complications
Answer: A
Subject: Primary and Secondary Glomerular Diseases

You have been asked to give a presentation to your colleagues in primary care on lupus nephritis, with emphasis on the presentation of lupus nephritis, interpretation of the serologic and histologic evaluation, and recommendations for follow-up testing..
Question: In the evaluation of lupus nephritis, which of the following is correct?
1. Observational studies suggest that lupus nephritis is being diagnosed at a younger age
2. The time from diagnosis of SLE to the development of lupus nephritis is shorter in African Americans and other minority groups
3. In patients with proliferative lupus nephritis who undergo repeated biopsy, histologic ﬁndings correlate closely with clinical response to treatment
4. Anti-PLA2R antibodies are occasionally identiﬁed in patients with class V lupus nephritis
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 28-year-old woman presents to her primary care provider with joint pain, fatigue, and a malar rash. Her laboratory evaluation shows positive ANA and anti–double stranded DNA antibodies; urinalysis shows 1+ blood and 2+ protein; serum creatinine is 0.8 mg/dL. Her biopsy specimen reveals class IV lupus nephritis, and you plan to start immunosuppressive therapy. You recommend starting mycophenolate mofetil and steroids, but she asks about alternative options and about recent developments in treatment..
Question: Which of the following is correct regarding the treatment of lupus nephritis?
1. The LUNAR trial showed improved outcomes from the addition of rituximab to mycophenolate mofetil and steroids
2. Post hoc analyses suggest that patients who achieve full depletion of peripheral B cells may gain greater beneﬁt from rituximab
3. Calcineurin inhibitors have shown little beneﬁt in the treatment of lupus nephritis
4. The addition of voclosporin to treatment with mycophenolate and steroids was associated with rates of remission, and adverse events that were similar to those of mycophenolate and steroids alone
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 55-year-old man is referred for evaluation of CKD, proteinuria, and new hematuria. He is HIV positive and has been taking antiretroviral therapy, including tenofovir disoproxil fumarate, for > 20 years with undetectable viral load. He received a diagnosis of type 2 diabetes and mild hypertension 10 years ago. He has 10 – 20 RBCs and 5 – 10 WBCs per high powered ﬁeld on urine microscopic examination, 2 grams of protein on 24-hour collection, and creatinine 2.2 mg/dL (eGFR 40 mL/min per 1.73 m2)..
Question: Which of the following statements is correct?
1. His antiviral medications should be left unchanged because they have been eﬀective in controlling HIV for over 20 years
2. Kidney biopsy will most likely show HIV-associated nephropathy
3. A stone should be excluded because tenofovir disoproxil fumarate commonly causes nephrolithiasis
4. He is at risk for immune complex glomerulonephritis that may or may not be related to his HIV
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 64-year-old woman who is new to the medical system recently received a diagnosis of hepatitis C virus (HCV) infection. She was referred to you because CKD screening performed as part of her HCV evaluation demonstrated creatinine 1.9 mg/dL, 2+ proteinuria, and 2+ hematuria. She feels well and has virtually no symptoms. She has not yet been prescribed any therapy..
Question: Which of the following statements is accurate?
1. A direct-acting antiviral (DAA) regimen, such as sofosbuvir and velpatasvir, and rituximab should be started concurrently because she has an active glomerulonephritis
2. She should be treated with rituximab ﬁrst, followed by any DAA
3. She should be treated with a DAA but not sofosbuvir because her eGFR is < 30 mL/min per 1.73 m2
4. She should be treated with a pangenotypic DAA irrespective of her eGFR, and rituximab should not be used, at least initially
Answer: D
Subject: Primary and Secondary Glomerular Diseases

You are consulted for 3+ proteinuria and AKI in a 39-year-old man who is admitted for severe unilateral ﬂank pain. He is an active user of both IV cocaine and heroin, and he is known to be hepatitis B virus positive but has refused treatment in the past. On examination he is very uncomfortable but has no point tenderness over either ﬂank. Urinalysis shows 2+ hematuria; leukocyte esterase is negative, with sediment showing nondysmorphic RBCs and few WBCs. Serum creatinine is 1.2 mg/dL. A 24-hour urine collection demonstrated 12 grams protein. Abdominal imaging shows normal kidneys, no obstruction, and no stone..
Question: What is the highest priority “next intervention”?
1. Perform a kidney biopsy to diagnose a glomerular cause
2. Treat for presumed urinary tract infection
3. Arrange for CT venography to exclude renal vein thrombosis
4. Treat with lamuvidine for presumed HBV-associated MN
Answer: C
Subject: Primary and Secondary Glomerular Diseases

Your patient is a 49-year-old HIV+ man with a 10-year history of hypertension, diabetes, and stage 4 CKD. He asks you about the risks and beneﬁts of transplantation..
Question: Which of the following statements is true regarding transplantation?
1. He would do better with transplantation than with dialysis, but time on the waiting list will be much higher than if he were HIV negative
2. He would do better with dialysis because the immuno-suppressive medications required for transplantation would probably activate HIV replication
3. He should do ﬁne with transplantation because the risks of transplantation are identical to those of HIV-negative patients, including the risk of rejection
4. He would do better with transplantation than with dialysis and should be referred for evaluation
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 55-year old woman is referred for evaluation of creatinine of 1.8 mg/dL with negative urinalysis results. By her report she has been in very good health except for “borderline diabetes” treated with metformin and diet. Creatinine was noted to be elevated in the setting of a viral illness before she underwent a chest CT-scan. Prior creatinine was approximately 1 year ago and was within normal range. She has no history of retinopathy. She has her urine checked at least twice yearly, and she thinks the results were always normal. Biopsy demonstrates GBM thickening, severe mesangial expansion, and nodular sclerosis in addition to mild acute tubular necrosis (ATN). The creatinine is down to 1.4 mg/dL by the time the biopsy is performed..
Question: Which of the following statements is likely to be true regarding the case presentation?
1. The patient is an inaccurate historian, and the diabetes was likely more severe and with accompanying retinopathy
2. Her primary care physician is not screening adequately for microalbuminuria, which always accompanies histologic changes of diabetic nephropathy
3. The biopsy was mislabeled and should be repeated
4. In addition to mild ATN from the viral syndrome, she has diabetic nephropathy with histologic changes that preceded clinical evidence such as eGFR decline or albuminuria
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 23-year-old man who is studying for his master’s degree in microbiology self-refers for evaluation of kidney function be-cause of a recent diagnosis of type 1 diabetes. He asks you to explain glomerular hyperﬁltration. Which of the following statements is FALSE regarding hyperﬁltration?.
Question: Select one:
1. Glomerular hyperﬁltration occurs within 1 to 5 years in most individuals with type 1 diabetes and is associated with structural lesions that predict a decline in GFR
2. Glomerular hyperﬁltration predicts cardiovascular mortality in type 2 diabetes
3. The sodium glucose cotransporters increase sodium delivery to the macula densa, which decreases GFR
4. Hyperglycemia and hyperaminoacidemia are initiating factors in altering glomerular hemodynamics
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 48-year-old man is referred for management of diabetic nephropathy. He has hypertension, hyperlipidemia, and atherosclerotic cardiovascular disease. His HbA1C is 8.5%, microalbumin/creatinine ratio is 480 mg/g, and eGFR 48 mL/min per 1.73 m2. He is taking metformin, lisinopril, hydrochlorothiazide, and atorvastatin..
Question: Which of the following is NOT associated with SGLT2 inhibitors?
1. Increased risk of genital fungal infections
2. Decreased risk of progression to ESKD
3. Increased risk of diabetic ketoacidosis
4. Increased cardiovascular mortality risk
Answer: D
Subject: Primary and Secondary Glomerular Diseases

You are following up a 50-year-old woman with longstanding hypertension, stage 3 CKD, and a recent diagnosis of diabetes. She is taking metformin, and her HbA1C is slightly above target. Her eGFR is 40 mL/min per 1.73 m2..
Question: Which of the following treatment options is most correct?
1. Stop the metformin and start an SGLT2 inhibitor
2. Continue the metformin and start an SGLT2 inhibitor
3. Stop the metformin and start insulin and an SGLT2 inhibitor
4. Continue the metformin and start glucagon-like peptide-1 receptor agonist
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 71-year-old man with a history of monoclonal gammopathy of undetermined signiﬁcance is referred for nephrology evaluation. His last bone marrow biopsy was 3 years prior and showed 10% kappa-restricted plasma cells. He has had slowly progressive CKD, with a recent serum creatinine of 1.8 mg/dL, which was 1.4 mg/dL 5 years before his current evaluation. He is also noted to have urine study results showing glycosuria with normoglycemia and a serum bicarbonate of 17 meq/L..
Question: Based on the clinical presentation, what would be the most likely histologic diagnosis if the patient underwent kidney biopsy?
1. Light chain proximal tubulopathy
2. Light chain deposition disease
3. AL (light chain) amyloidosis
4. Light chain cast nephropathy
Answer: A