Polycystic Kidney Disease, Alport & Goodpasture: Step 1 review notes and self-test
Notes for the Polycystic Kidney Disease, Alport & Goodpasture rapid review: the clue that separates each of its 5 confusion pairs, with timestamps and a self-test.
October 21, 2026
This video covers polycystic kidney disease, Alport syndrome, diabetic nephropathy, Goodpasture syndrome, and related look-alike conditions. Watch the video, then use these notes to test yourself on the clue that separates each pair.
Autosomal dominant polycystic kidney disease vs Autosomal recessive polycystic kidney disease
Use age first, then the liver pattern. ADPKD causes adult-onset bilateral enlarged cystic kidneys with hepatic cysts and berry aneurysm risk, while ARPKD presents in infancy or childhood with enlarged echogenic kidneys and congenital hepatic fibrosis or portal hypertension. Both can enlarge the kidneys and involve the liver, so remember cysts for ADPKD and congenital fibrosis for ARPKD.
Autosomal dominant polycystic kidney disease vs Autosomal recessive polycystic kidney disease ADPKD is adult cystic kidney disease with aneurysm/liver cyst clues; ARPKD is infantile kidney-liver fibrocystic disease. Open the comparison and video →Alport syndrome vs Thin basement membrane disease / IgA nephropathy
Sort recurrent hematuria by the associated clue. Alport syndrome adds hearing loss, ocular abnormalities, and progressive kidney disease, while thin basement membrane disease causes benign isolated microscopic hematuria and IgA nephropathy causes synpharyngitic gross hematuria with normal complement. Young age and hematuria alone do not separate them.
Alport syndrome vs Thin basement membrane disease / IgA nephropathy Alport is hematuria plus ear/eye/progression; thin basement membrane is benign isolated hematuria; IgA is synpharyngitic hematuria. Open the comparison and video →Diabetic nephropathy vs Renal amyloidosis
Look for diabetic microvascular disease versus systemic amyloid clues. Long-standing diabetes, retinopathy, and nodular glomerulosclerosis support diabetic nephropathy, while Congo red-positive deposits or systemic findings such as restrictive cardiomyopathy, neuropathy, hepatosplenomegaly, plasma-cell dyscrasia, or chronic inflammation support renal amyloidosis. Do not assume every nephrotic syndrome in a patient with diabetes is diabetic nephropathy, especially when it develops unusually quickly.
Diabetic nephropathy vs Renal amyloidosis Diabetic nephropathy is diabetes-duration nodular sclerosis; amyloidosis is Congo red-positive systemic protein deposition. Open the comparison and video →Goodpasture syndrome vs Granulomatosis with polyangiitis
Use ENT involvement to separate these pulmonary-renal syndromes. Goodpasture syndrome causes pulmonary hemorrhage and rapidly progressive GN with anti-GBM antibodies and linear IgG, while GPA adds sinus disease, persistent otitis media, nasal septal perforation, saddle nose, lung nodules or cavitation, and c-ANCA or PR3 positivity with pauci-immune crescentic GN. Hemoptysis plus hematuria alone is not enough to choose between them.
Goodpasture syndrome vs Granulomatosis with polyangiitis Goodpasture: pulmonary-renal with anti-GBM and no ENT disease; GPA: adds sinusitis and saddle nose with c-ANCA/PR3. Open the comparison and video →Atherosclerotic renal artery stenosis vs Fibromuscular dysplasia
Use the age, sex, and imaging pattern. An older patient with vascular disease and ostial or proximal stenosis supports atherosclerotic renal artery stenosis, while a younger woman with mid-to-distal string-of-beads supports fibromuscular dysplasia. Hypertension and a renal bruit can occur in both, and a creatinine rise above 30 percent after an ACE inhibitor or ARB can suggest bilateral renal artery disease in either case.
Atherosclerotic renal artery stenosis vs Fibromuscular dysplasia Older vascular-risk patient means atherosclerosis; young woman with string-of-beads means fibromuscular dysplasia. Open the comparison and video →Quick self-test
Name the diagnosis from the clue, then tap to check.
Sinus disease, lung nodules or cavitation, renal disease, and c-ANCA/PR3 positivity.
Granulomatosis with polyangiitis · Goodpasture syndrome vs Granulomatosis with polyangiitis
Pulmonary hemorrhage plus rapidly progressive GN with anti-GBM antibodies.
Goodpasture syndrome · Goodpasture syndrome vs Granulomatosis with polyangiitis
Inherited collagen IV disease with persistent hematuria, progressive kidney disease, sensorineural hearing loss, and ocular abnormalities.
Alport syndrome · Alport syndrome vs Thin basement membrane disease / IgA nephropathy
Long-standing diabetes with albuminuria, hypertension, progressive CKD, retinopathy support, and nodular glomerulosclerosis.
Diabetic nephropathy · Diabetic nephropathy vs Renal amyloidosis
Nephrotic proteinuria with systemic amyloid clues such as restrictive cardiomyopathy, neuropathy, hepatosplenomegaly, plasma-cell dyscrasia, chronic inflammation, or Congo red-positive deposits.
Renal amyloidosis · Diabetic nephropathy vs Renal amyloidosis
Older patient with vascular disease, resistant hypertension, abdominal bruit, or creatinine rise after ACE inhibitor.
Atherosclerotic renal artery stenosis · Atherosclerotic renal artery stenosis vs Fibromuscular dysplasia
Younger woman with renovascular hypertension and string-of-beads renal artery appearance.
Fibromuscular dysplasia · Atherosclerotic renal artery stenosis vs Fibromuscular dysplasia
Keep going
Every pair in this review has a free page in the library with a side-by-side comparison and a practice question. Work through the ones you missed in the self-test first.