SeanWu25/NEJM-AI_Benchmarking_Medical_Language_Models huggingface dataset:
Subject: Primary and Secondary Glomerular Diseases

A 62-year-old woman presents with a 6-week history of systemic illness, with fatigue, weight loss, intermittent fevers, and bloody nasal discharge. She is found to have AKI, with creatinine 2.3 mg/dl, and new-onset hematuria and proteinuria. Additional laboratory tests demonstrate anemia (hemoglobin 10.2 g/dL), thrombocytosis (platelet count 523,109/L), eosinophilia (count 900 cells per µL), and elevated C-reactive protein (81 mg/dl). Chest radiography reveals bilateral cavitation in the lungs..
Question: Regarding the diagnosis of ANCA-associated vasculitis in this patient, which of the following statements is MOST correct?
1. Indirect immunoﬂuorescence is the preferred screening test for detection of circulating ANCA
2. Positive testing for MPO-ANCA excludes a diagnosis of granulomatosis with polyangiitis
3. The demonstration of granulomatous inﬂammation on tissue biopsy is required to conﬁrm a diagnosis of GPA
4. GN is more common in microscopic polyangiitis than in GPA
5. Serum-soluble CD163 is a sensitive and speciﬁc biomarker of GN in patients with both microscopic polyangiitis and GPA
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 42-year-old man is admitted to the hospital with malaise, nausea, dyspnea, and oligoanuria. His serum creatinine is elevated at 7.1 mg/dl. He is initiated on acute KRT. He is found to be MPO-ANCA positive, and kidney biopsy conﬁrms a diffuse crescentic GN, with a paucity of immune deposits..
Question: Regarding remission induction treatment for this presentation of ANCA-associated GN, which of the following statements is MOST correct?
1. A recent meta-analysis has shown that plasma exchange has no effect on renal outcomes in ANCA-associated GN
2. A reduced dose glucocorticoid regimen is equally effective as a standard dose regimen and associated with fewer infections in patients treated with cyclophosphamide
3. Avacopan is superior to glucocorticoids for achieving sustained disease remission at 6 months
4. A randomized, controlled trial has shown that daily oral cyclophosphamide and rituximab infusions are equally effective for the induction of remission
5. Treatment with mycophenolate mofetil is associated with fewer infections than with cyclophosphamide
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 37-year-old woman was diagnosed with PR3-ANCA–associated vasculitis 6 months ago and was treated with rituximab and prednisone for induction therapy. She is currently in clinical remission (BVAS=0). Testing for ANCA became negative within 3 months of initiating therapy, and her peripheral B cell count was zero at the 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 bene-ﬁts of azathioprine versus rituximab..
Question: Regarding remission maintenance therapy in AAV, which of the following is MOST correct?
1. In a clinical trial comparing it with maintenance therapy with azathioprine, rituximab resulted in more infections during the maintenance of remission of AAV
2. A rituximab dosing strategy on the basis of repopulation of peripheral B cells and/or changes in ANCA titer led to fewer major relapses and longer relapse-free survival than the 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. Prolonged maintenance treatment with rituximab for up to 3 years is associated with a lower relapse rate and similar incidence of serious adverse events com-pared with maintenance treatment for 18 months
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 27-year-old man who is a cigarette smoker presents with pulmonary-renal syndrome. A kidney biopsy shows a diffuse crescentic GN, with linear deposits of IgG and C3 on immunostaining..
Question: Regarding the serologic assessment of anti-GBM disease, which of the following statements is MOST correct?
1. The detection of circulating anti-GBM antibodies is essential for diagnosis
2. The detection of ANCA does not inﬂuence treatment or outcome
3. Antibodies to laminin-521 are found more frequently in patients with lung hemorrhage
4. Peroxidasin antibodies are found more frequently in patients with severe kidney disease
5. Anti-GBM antibodies are detected in up to 5% of patients with SLE
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 62-year-old woman presents with a 1-week history of fatigue and a 3-day history of dark urine and dys-pnea, with reduced urine output within the last 24 hours. On examination, her BP is 182/98 mm Hg, and the examination is notable for JVD, bibasal rales, and 1+ lower limb edema. Chest radiography reveals bilateral ground glass change with apical and peripheral sparing. Her creatinine is 6.8 mg/dl, and she is oliguric. Her urine sediment shows numerous RBCs, including many acanthocytes, and RBC casts. She undergoes renal biopsy, which shows crescentic GN affecting all glomeruli, and linear staining for IgG on immunoﬂuorescence consistent with anti-GBM dis-ease. Her serologic workup reveals dual positivity for ANCA and anti-GBM antibodies..
Question: Regarding the treatment and outcome of anti-GBM disease in this patient, which of the following is MOST correct?
1. The presence of lung hemorrhage is associated with poorer renal outcome
2. Treatment with daily oral cyclophosphamide is preferred to pulse intravenous dosing
3. Following treatment with plasma exchange, cyclo-phosphamide, and glucocorticoids, recovery from dialysis-dependent renal failure can be expected in approximately 30% of cases
4. A randomized, controlled trial has shown that imliﬁdase is superior to plasma exchange for the rapid removal of anti-GBM antibodies from circulation
5. Maintenance immunosuppression is not required
Answer: B
Subject: Primary and Secondary Glomerular Diseases

You have been asked to give a presentation to your colleagues in primary care on lupus nephritis, with an emphasis on the presentation of lupus nephritis, the 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 MOST 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 Blacks and other minority groups
3. In patients with proliferative lupus nephritis who undergo rebiopsy, histologic ﬁndings correlate closely with clinical response to treatment
4. Anti-PLA2R antibodies are frequently identiﬁed in patients with class 5 lupus nephritis
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 28-year-old woman presents to her primary care physician with joint pain, fatigue, and a malar rash. Her laboratory evaluation shows positive ANA and anti-dsDNA antibodies; urinalysis shows 1+ blood and 2+ protein, and serum creatinine is 0.8 mg/dl. Her biopsy reveals class 4 lupus nephritis, and you plan to start her on immunosuppression. You recommend starting mycophenolate mofetil and steroids, but she asks about alternative options and recent developments in treatment..
Question: Which of the following statements is supported by the ﬁndings of recent prospective, randomized, con-trolled studies?
1. Rituximab is an effective treatment for lupus nephritis
2. Treatment with obinutuzumab, in addition to standard care, improves renal responses but is associated with a higher risk of infection
3. Belimumab, in addition to standard therapy, has demonstrated efﬁcacy in the treatment of lupus nephritis and extrarenal manifestations of SLE
4. Calcineurin inhibitors, such as voclosporin, are an effective alternative to mycophenolate mofetil for the treatment of proliferative lupus nephritis
5. The addition of anifrolumab to standard therapy results in greater improvements in proteinuria in patients with high type 1 IFN gene signatures
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 44-year-old morbidly obese Hispanic man who has not received COVID-19 vaccination is admitted to the hospital with respiratory distress 8 days after the initial onset of rhinorrhea and cough. In the emergency room, he is hypoxic and requires high-ﬂow oxygen, and he is started on remdesivir and dexamethasone. His hypoxia worsens over the next 48 hours, and he is transferred to the intensive care unit. His admission creatinine was 1.2 mg/dl (eGFR576 ml/min per 1.73 m2) and has risen to 2.5 mg/dl on hospital day 3. His urinalysis shows 1+ blood and 1+ protein. On hospital day 5, his creatinine has risen to 4.6 mg/dl, and his nephrology team recommends starting continuous KRT..
Question: Which of the following statements is MOST correct?
1. Remdesivir is the most likely cause of his AKI
2. A kidney biopsy would most likely show thrombotic microangiopathy
3. The mortality of stage 3 AKI in patients admitted to the intensive care unit with COVID-19 exceeds 50%
4. AKI most commonly occurs in the ﬁrst 5 days of COVID-19 illness
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 36-year-old Black woman presents to her primary care doctor due to a 15-pound weight gain and lower extremity edema over the last 7 days. She was well until 3 weeks ago, when she developed a sore throat and cough, and she was diagnosed with COVID-19 by RT-PCR test; her COVID-19 symptoms were mild and lasted 8 days, and she felt fully recovered until she noted leg swelling. Her laboratory studies are notable for a creatinine of 2.9 (unknown baseline), and 24-hour urine collection demonstrates 5.5 g of proteinuria and 4.0 g of albuminuria. Urine microscopy shows ﬁve to 10 RBCs and zero to ﬁve WBCs..
Question: Which of the following statements is MOST correct?
1. There are numerous glomerular diseases that more commonly occur in patients with COVID-19 com-pared with controls, including COVID-19–associated collapsing glomerulopathy, minimal change disease, MN, and pauci-immune GN
2. COVID-19–associated collapsing glomerulopathy has been described in patients with high-risk APOL1 genotypes
3. The pathogenesis of COVID-19–associated collapsing glomerulopathy involves direct viral infection of podocytes
4. Early antiviral therapy has been shown to improve outcomes in COVID-19–associated col-lapsing glomerulopathy
5. COVID-19–associated collapsing glomerulopathy typically only occurs in patients with severe or critical COVID-19
Answer: B
Subject: Primary and Secondary Glomerular Diseases

A 64-year-old man is referred for evaluation of CKD and new-onset nephrotic-range proteinuria. He is HIV positive and has been taking antiretroviral therapy for >10 years with undetectable viral load. His current antiretroviral regimen is bictegravir, emtricitabine, and tenofovir alafenamide. His other medication problems include hypertension diagnosed at age 45 well controlled on hydrochlorothiazide 25 mg daily. He has zero to ﬁve RBCs and zero to ﬁve WBCs per high-powered ﬁeld on urine microscopic examination, 4.6 g of protein on 24-hour collection, and creatinine of 1.2 mg/dl (eGFR575 ml/min per 1.73 m2). Kidney biopsy shows membranous glomerulopathy. Anti-PLA2R antibody titer has been sent and is not yet back..
Question: Which of the following statements is MOST correct?
1. He does not need to be screened for malignancy
2. His antiviral medications should be changed
3. His biopsy will most likely stain for THSD7 antibody
4. Treatment of HIV alone is unlikely to lead to full or partial remission of kidney disease
Answer: D
Subject: Primary and Secondary Glomerular Diseases

A 70-year-old Black woman is referred for evaluation of CKD and newly detected proteinuria. She was diagnosed with AIDS in 1996 when she presented with a CD4 count of ten; she has been taking antiretroviral therapy for > 25 years with undetectable viral load. Her antiretroviral regimen is efavirenz, emtricitabine, and tenofovir disaproxil fumarate. Her other medication problems include HCV genotype 2 infection that was cured 5 years ago using sofosbuvir and ribavirin and hypertension diagnosed at age 60 that is well controlled with hydrochlorothiazide 25 mg daily and lisinopril 10 mg daily. Her examination shows normal vital signs, BMI of 18, and temporal wasting. She has ten to 20 RBCs and zero to ﬁve WBCs per high-powered ﬁeld on urine microscopic examination. She has a 24-hour urine collection showing 1.1 g of protein and 180 mg of albuminuria. Her serum creatinine is 1.3 mg/dl (eGFR544 ml/min per 1.73 m2), and her serum phosphorus level is 1.9 mg/dl..
Question: Which of the following statements is MOST correct?
1. Her antiviral medications should be left un-changed because they have been effective in controlling HIV for over 20 years
2. Kidney biopsy is most likely to show immune com-plex GN that may or may not be related to HIV
3. Kidney biopsy will most likely show HIVAN
4. PLA2R levels can rule out MN
5. Cessation of tenofovir disoproxil fumarate will lead to improvement in proteinuria and hypophosphatemia
Answer: E
Subject: Primary and Secondary Glomerular Diseases

A 62-year-old woman receives a diagnosis of HCV infection after routine screening by her primary care doctor. She was referred to you because CKD screening performed as part of her HCV evaluation demonstrated creatinine 1.4 mg/dl, 2+ proteinuria, and 2+ hematuria. When evaluated in your ofﬁce, she has normal vital signs; her repeat laboratory studies con-ﬁrm a creatinine of 1.4, and 24-hour proteinuria collection demonstrates 2.9 g of proteinuria. Her cryocrit level is 2%. She undergoes a kidney biopsy showing membranoproliferative GN with histologic activity but no crescents. She feels well and is asymptomatic. She has not yet been prescribed any therapy..
Question: Which of the following statements is MOST accurate?
1. A direct-acting antiviral (DAA) regimen, such as sofosbuvir and velpatasvir, and rituximab should be started concurrently because she has an active GN
2. She should be treated with a nonhepatically metabolized DAA regimen because of her kidney disease
3. She should be treated with rituximab ﬁrst followed by any DAA
4. She should undergo plasmapheresis, rituximab, corticosteroids, and DAA therapy
5. She should be treated with a pangenotypic DAA, and rituximab should not be used, at least initially
Answer: E
Subject: Primary and Secondary Glomerular Diseases

A 14-year-old boy is admitted for severe unilateral ﬂank pain. He was perinatally infected with hepatitis B and is known to be hepatitis B virus positive, but he has had poor compliance with antiviral therapy. On examination, he is uncomfortable, writhing in bed, but he has no costoverterbral angle tenderness. Urinalysis shows 4+ proteinuria and 3+ hematuria; leukocyte esterase is negative, with sediment showing ﬁve to ten nondysmorphic RBCs and zero to ﬁve WBCs. Serum creatinine is 0.9 mg/dl. A 24-hour urine collection demonstrated 14 g protein. Abdominal imaging shows normal kidneys, no obstruction, and no stone..
Question: What is the next BEST step in his management?
1. Perform a kidney biopsy to diagnose the cause of nephrotic syndrome
2. Treat with intravenous antibiotics for presumed pyelonephritis
3. Exclude renal vein thrombosis with appropriate imaging
4. Begin antiviral therapy for presumed hepatitis B virus–associated MN
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 61-year-old man with HIV infection who has stage 5 CKD (attributed to long-standing hypertension and diabetes) is referred for transplant evaluation..
Question: Which of the following statements is MOST COR-RECT regarding transplantation?
1. He would do better with dialysis than transplantation because the immunosuppressive medications required for transplantation would lead to loss of HIV control
2. There is no need to look for a live donor because he can get an HIV-infected deceased donor very rapidly
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 will need to avoid calcineurin inhibitors after transplant due to interactions with antiretroviral therapy for HIV
5. Providing that institutional safeguards have been established and the patient’s HIV is controlled with antiretroviral therapy, he is a good candidate for transplantation
Answer: E
Subject: Primary and Secondary Glomerular Diseases

A 45-year-old man with type 2 diabetes mellitus has been referred to you for further evaluation. On review of his medical record, you observe that he was diagnosed with diabetes 5 years earlier and has lost > 12 ml of GFR over the last 2 years. You advise him that a kidney biopsy is required to exclude the diagnosis of an alternative nondiabetic kidney disease to explain his progressive GFR loss. He asks you to explain the risks associated with kidney biopsy. The largest meta-analysis of clinically indicated kidney biopsy complications to date emanated from the Kidney Precision Medicine Program..
Question: On the basis of these data, which ONE of the following is correct?
1. Death does not occur as a result of kidney biopsy
2. Complication rates are equivalent in outpatient and inpatient kidney biopsy procedures
3. The need for intervention to halt excessive bleeding from a clinically indicated kidney biopsy occurs in three of 1000
4. Gross hematuria from a clinically indicated kidney biopsy is extremely rare, occurring in <1%
Answer: C
Subject: Primary and Secondary Glomerular Diseases

A 63-year-old woman with type 2 diabetes mellitus and stage A3G3a (baseline creatinine 1.3 mg/dl) is hospitalized for a urinary tract infection and AKI. Her metformin and valsartan were both stopped; her creatinine peaked at 4.2 mg/dl but was down to 2.8 mg/dl on discharge. You are seeing her 2 weeks later for a posthospital follow-up. Her BP is 128/76 mm Hg, heart rate is 72 bpm, creatinine is 1.7 mg/dl (eGFR is 33 ml/min per 1.73 m2), hemoglobin A1c is 7.6%, and UACR is 480 mg/g. Her discharge medications include sitagliptin, amlodipine, carvedilol, and simvastatin..
Question: What is the BEST medication change for her long-term cardiovascular and kidney health?
1. Start her on an SGLT2 inhibitor
2. Restart her angiotensin receptor blocker
3. Restart her on metformin
4. Start her on a GLP1 agonist
Answer: B
Subject: Primary and Secondary Glomerular Diseases

You are seeing a 46-year-old man for follow-up after his initial consultation for CKD stage A3G4 in the setting of type 2 diabetes mellitus. He has a history of coronary artery disease and had a stent of his right coronary artery last year. His urine sediment, serologies, and ultrasound were all unremarkable. Today, his vital signs show a BP of 136/84 mm Hg, heart rate of 80 bpm, and BMI of 42. His hemoglobin A1c is 7.6%, creatinine is 3.0 mg/dl (eGFR526 ml/min per 1.73 m2), UACR is 280 mg/g, and potassium is 4.6 mEq/dl. His medications include insulin glargine 120 units nightly, A spart insulin 22 units with meals, empagliﬂozin 10 once mg daily, losartan 100 mg once daily, amlodipine 10 mg once daily, carvedilol 25 mg once daily, and chlorthalidone 50 mg once daily. You discuss goals for nutrition and exercise..
Question: What is the BEST medication change you can make for his overall medical prognosis?
1. Increase his empagliﬂozin to 25 mg daily
2. Change his losartan 100 mg daily to valsartan 320 mg daily
3. Add semaglutide starting at a dose of 0.5 mg subcutaneously once weekly
4. Add ﬁnerenone 10 mg daily
Answer: C
Subject: Disorder of Divalent Ions Renal Bone Disease and Nephrolithiasis

A 72-year-old hemodialysis patient is found to have a plasma phosphate of 9.0 mg/dl on routine monthly lab testing. He takes calcitriol 0.5 mcg daily for treatment of secondary hyperparathyroidism but reports noncompliance with phosphate binders and dietary phosphate restriction. He is educated regarding the need to comply and is referred to meet with a nutritionist at his hemodialysis unit to reduce dietary phosphate intake..
Question: Which of the following is MOST CORRECT regarding intestinal phosphate absorption?
A.        The activity of Pit-1 and Pit-2 is increased by calcitriol.
B.        Dietary phosphate within phytic acid is readily absorbed owing to the widespread activity of phytases in the intestine.
C.        Tenapanor inhibits the Na+ /H+ -exchanger isoform 3 (NHE3) in the intestine, thereby reducing paracellular phosphate transport.
D.        The phosphate transporter NaPillb is expressed across the entire small and large intestine.
Answer: C
Subject: Disorder of Divalent Ions Renal Bone Disease and Nephrolithiasis

A liver transplant recipient with hypophosphatemia (serum phosphate, 1.0 mg/ dl) is started with phosphate supplementation. His medical history, lab data, and medications are reviewed to identify a cause..
Question: Which is the BEST test to investigate the underlying cause?
A.        Measure fractional phosphate excretion
B.        Measure tubular maximal reabsorption of phosphate/ glomerular filtration rate (TmP/GFR) in a second morning urine after an overnight fast
C.        Determine serum FGF23 and PTH concentrations
D.        Determine trough levels of immunosuppressants
Answer: B
Subject: Disorder of Divalent Ions Renal Bone Disease and Nephrolithiasis

A 52-year-old woman with type 2 diabetes, hypertension, and dyslipidemia is following up with her primary care physician (PCP). Routine labs reveal her eGFR is 70 ml/min per 1.73 m2, with albuminuria 40 mg/ g on spot urine sample and plasma phosphate 4.3 mg/ dl (upper limit of normal, 4.5 mg/ dl)..
Question: Which of the following is MOST CORRECT regarding hyperphosphatemia?
A. In the general population, plasma phosphate levels at the upper end of the normal range have been associated with increased cardiovascular mortality
B.        Once eGFR levels decrease below 60 ml/min per 1.73 m2, fibroblast growth factor (FGF)-23 levels increase to protect against hyperphosphatemia
C.        Metabolic or respiratory alkalosis is associated with hyperphosphatemia
D.        Dietary phosphate in food additives is poorly bioavailable
Answer: A
Subject: Disorder of Divalent Ions Renal Bone Disease and Nephrolithiasis

A 68-year-old woman presents to the emergency department with a 5-month history of gradually progressive fatigue, lower extremity edema, and headaches. She has not been seen by a physician for more than 15 years and is taking no medications. BP on arrival is 180/110 mm Hg, and her creatinine level is 5 mg/dl. Her kidneys are small and echogenic on ultrasound, and an electrocardiogram shows left ventricular hypertrophy. Of note, her serum calcium is 7 mg/ dl, ionized calcium is 0.9 mmol/L, phosphate is 6.5 mg/ dl, and PTH is 465 pg/ml..
Question: Which of the following is NOT a direct action of PTH?
A.        Increased intestinal absorption of calcium
B.        Increased activity of lα-hydroxylase
C.        Increased tubular reabsorption of calcium
D.        Increased bone resorption
Answer: A
Subject: Disorder of Divalent Ions Renal Bone Disease and Nephrolithiasis

A 5-year-old boy was referred to a renal pediatric service because of recurrent urinary tract infections. Labo­ratory findings were notable for eGFR creatinine of 65 ml/min per 1.73 m2, hypomagnesemia (1.1 mg/dl; reference range, 1.6-2.6 mg/ dl), elevated fractional excretion of magnesium (FeMg, 12%; reference range, < 4%), hypercalciuria (12 mg/kg per day; reference range, < 4 mg/kg per day), hypocitraturia (120 mg/ d; reference range, 300-600 mg/ d), and elevated serum PTH (110 pg/ml; reference range, 10-65 pg/ml). On ultrasound, nephrocalcinosis was observed. His parents were cousins, and his 20-year-old brother had recently a diagnosis of advanced CKD..
Question: Which ONE of the following genetic abnormalities is MOST likely causing this clinical condition?
A.        Gitelman syndrome
B.        Distal renal tubular acidosis
C.        Bartter syndrome
D.        A loss-of-function mutation in the CLDN16 gene
Answer: D