ABIM Nep GNs & Renal Diseases
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| Alpha-1 antitrypsin is assoc with which ANCA? | anti-PR3 but not anti-MPO
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| Describe IgG4-related disease. | Marked plasma cell infiltrate (tubulointerstitial nephritis) + autoimmune pancreatitis, but all organs affected. IgG4 deposited in the tubules. ++activate complement. May see eosinophilia and low+ANA , MGN, obstruction . pulm and renal masses.
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| What disease presents with picture of low complements, AIN, renal/lung masses, low grade fever? | IgG4-related systemic disease.
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| What disease presents with picture of pulmonary, eye, skin, and renal involvement, along with renal biopsy findings of a cellular infiltrate and granuloma? | sarcoidosis
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| What disease presents with picture of dry eye, sicca syndrome, and interstitial nephritis (without granuloma) +/- systemic inflammation? | autoimmune dz of Sjögren’s syndrome. Usu no glomerular dz.
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| What disease presents with picture of uveitis + interstitial nephritis (sometimes granulomas), fanconi syn? | TINU. idiopathic IAN + ant uveitis usu in adolescent girls.
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| What disease presents with picture of |
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| von Hippel-Lindau syndrome abnormalities include? | cysts in the kidneys, pancreas, and genital tract. increased risk of developing clear cell renal cell carcinoma and pancreatic neuroendocrine tumor & pheochromocytoma
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| What disease presents with skin papule, cystic kidney disease, possible mental retardation? | Tuberous Sclerosis
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| When do you start IS for IgAN? | IgA nephropathy "with persistent proteinuria > 1 g daily, despite 3-6 months of med rx(ACE-I or ARBs & BP control), and GFR > 50 mL/min/1.73 m2, receive a 6-month course of corticosteroid therapy.”
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| Tumors assoc w tuberous sclerosis? | angiomyolipomas. AD inheritance Major bleeding rx’d w/ nephron-sparing surgery
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| How can mTORs help in tuberous sclerosis? | mTOR pathway, may be effective in markedly reducing the volume of target angiomyolipomas.
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| Thrombotic thrombocytopenic purpura is diagnosed with what lab test? | ADAMTS13 activity LESS THAN 5%
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| Albumin < ___ is the most significant independent predictor of venous thrombotic risk in NS? | 2.8 g/dL. Clin J Am Soc Nephrol. 2012;7:43-51
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| Rx IgAN? | 6-mo prednisone for persistent proteinuria >1 g/24 hr after a 3-6 therapeutic trial of RAAS ing & adequate BP control. Nephrol Dial Transplant. 2009; 24:3694-3701
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| Poor prognostic indicators for IgAN? | HTN, impaired GFR at presentation, and proteinuria. J Am Soc Nephrol. 2012;23:1753-1760
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| Abnormalities of alternative pathway cause what renal issues? | C3GN and DDD. Caused by factor H defect/deficiency or antibodies against alt complement pathway (aka C3 nephritic factor). assoc w low C3 and nl C4.
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| Rx C3GN? | Eculizumab. Two recent reports utilizing Ecu for DDD or C3 GN were assoc w stabilization of GFR and improved renal path or less proteinuria in some but not all patients. J Am Soc Nephrol. 2012;23:1229-1237
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| Nephrotic syndrome in inflammatory bowel disease caused by? | Secondary (AA) amyloidosis. Often assoc w/ orthostasis suggesting autonomic neuropathy, hematuria (fragile bladder mucosa),
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| Renal conditions w Crohn’s? | Mesalamine-induced AIN, calcium oxalate stones.
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| Most common histologic entity associated with adult-onset nephrotic syndrome in Caucasians? | Membranous nephropathy
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| Biopsy findings of membranous GN? | LM thickened capillary walls; PAS shows spikes; IF + IgG + complement. EM subepithelial deposits give “spike” appearance.
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| target for MGN rx? | partial remission (<3.5 g/d & 50% reduction from peak proteinuria). Partial remission is assoc w both significant slowing CKD progression & doubling of kidney survival at 10 years
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| Risk of progression from membranous GN | complete remissions in proteinuria occur in 20% to 30%. Low risk for progression if nl GFR & prot <4g. High-risk if proteinuria ≥8 g/d over 3 to 6 mo and/or declining CrCl during this observation period. medium risk if in between.
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| Rx for primary MGN | steroids + cytotoxic (cyclophosphamide, chlorambucil, MMF) for med risk. BUT high risk pt start w/ cyclosporine. Resistant cases-ACTHAR, rituximab,
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| Rx FSGS | prednisone 1 mg/kg max 60 g/d x 3-6 mos;
CsA 3-5 mg/kg/d divided BID x 6-12 mos;
tacrolimus 0.2-0.3 mg/kg/d divided BID x 6-12 mos;
MMF 25-35 mg/kg/d divided BID (max 2g/d) WITH dex.
[tac or CsA slow taper]
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| Distinguish thin BM dz vs Alport. | Thin BM dz has GBM that is thin throughout. Alports has some thick/thin areas.
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| Thin Basement membrane clinical features | min proteinuria, hematuria, nl GFR, benign course. May be carrier state of Alport.
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| Alport clinical features | persistent hematuria, proteinuria 1-2g/d, renal failure progressive, deafness, lenticonus, Leiomyomitosis, cognitive impairment, retinopathy. X-linked most common. Carriers look like thin BM.
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| Sub epithelial deposits on EM are classic of _____ GN. | membranous nephropathy, + PLA2R. Decline of anti-PLA2R titer precedes clinical response.
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| _______ is the first podocytic antigen responsible for human membranous nephropathy (MN) in adults. | Thrombospondin type-1 domain-containing 7A (THSD7A). Neutral endopeptidase (NEP) in neonates & cationic bovine serum albumin (cBSA in children.
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| cANCA pattern associated with ____ | PR3 (think c3po). Has higher rate of relapse
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| pANCA pattern associated with ____ | MPO (think c3po)
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| Microscopic Polyangiitis (MPA) | Pauciimune necrotizing vasculitis of small/medium arteries. Associated with pulmonary disease
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| Hemolytic uremic syndrome without diarrhea is known as _______ | atypical HUS. Could be from infections or complement deficiency (acquired/inherited).
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| T/F alternate pathway is constantly activated at low levels . | TRUE. It is usually turned off, but failure to down regulate (by Factor H 1, etc) results in unchecked complement activation.
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| C3GN | previously called MPGN. no IgG deposition or staining but ++C3 stain. Caused by alternative pathway dysregulation from abnormal proteins or antibodies.
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| C3nephritic factor present + DDD treatment is ______ | plasma exchange or infusion, Rituximab
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| CFH mutation present in DDD treatment is ______ | plasma infusion
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| T/F FSGS tip lesion has worst prognosis. | False since has best prognosis
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| T/F FSGS collapsing lesion has worst prognosis | TRUE. Maybe idiopathic or secondary to viral infections, medications
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| T/F FSGS NOS is rare | FALSE. It’s most common.
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| T/F FSGS cellular variant is rare | TRUE; it is the least common
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| Viruses associated w FSGS | HIV, parvo B19, HCV, CMV, EBV
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| Drugs assoc w FSGS | pamidronate, heroin, interferon, lithium, calcineurin inh
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| APOL1 associated with? | collapsing FSGS, HIVAN, sickle cell, SLE, focal global sclerosis (HTN). 2 risk variants assoc w 20% chance of developing renal dz.
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| Genetic testing for FSGS | usu not done, but NPHS2pR229Q is a permissive mutation that requires 2cnd mutation (compound heterozygotes) to cause FSGS. esp common in Western Europe.
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| Nail patella syndrome | AD loss of fcn mutation of LMX1B, assos w symmetric nail, skeletal, ocular and kidney disease. Biopsy + “whorling” pattern.
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| When is genetic testing for FSGS undertaken? | children w congenital NS, children w SRNS, familial FSGS, (sopradic FSGS consider R229Q NPHS2 variant)
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| AL-Type Amyloidosis histology | Renal, pul, GI & cardiac involvement most common. definitive dx congo red stain apple birefringence; amorphous eosinophilic nodular infiltrate in glom. IF + smudgy lambda>kappa. EM random oriented fibrils.
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| Immunotactoid acute tubulopathy looks like? | glomerular deposits, Large organized fibril in GBM.
IF + either kappa or lamb not both;
EM Tubular deposits > 30 nm
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| LCDD acute tubulopathy looks like? | fanconi + AKI + LC crystals (light blue on H&E). EM: LC crystals needle shaped, rectangular, rod-like and electron dense.
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| T/F Free light chains maybe elevated with low GFR. | true
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| The presence of free light chains indicates a biopsy when ____. | kappa/lambda ratio is skewed (ie normal is 0.26 - 1.6). Especially with tubulopathy and AKI.
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| Rituximab in ANCA-Associated Vasculitis (RAVE) trial showed | ritux + steroids had similar results to cyclophosphamide+steroids for induction of remission and may be superior in relapsing disease. Alveolar hemorrhage pts had same outcomes but relapsers fared better in ritux arm. Similar SAE noted.
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| LCCD histology | nodular glomerulsclerosis; IF makes dx staining for kappa>lambda; EM finely granular amorphous deposits
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| HIV assoc kidney disease? | HIVAN, MCD, postinfectious GN, amyloidosis, and IgAN. Coinfection with hepatitis B and C common—> MGN + MPGN
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| myeloma kidney presents with _______ | prox RTA (tubulopathy), acidic urine, usu kappa chains. UA trace pro, + glu, bland sediment. 24 hr urine ++ protein. Labs show nl glucose, low phos
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| AKI + back pain make you think of? | multiple myeloma
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| ESRD + back pain make you think of? | paraspinal abscess from bacteremia
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| What agent is contraindicated to treat/prevent TLS in G6PD deficient pts? | Rasburicase.
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| T/F Rasburicase.and allopurinol are equally effective to treat TLS. | TRUE
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| Role of allopurinol in TLS? | safe drug assuming good kidney function. Lowers further uric acid production but does NOT correct pre-existing hyperuricemia.
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| T/F alkalinizing urine is recommended for TLS. | NO. IV saline is best. watch if patient has AKI or vol overload. Increasing the urine pH decreases UA precipitation that enhances Ca-Ph precipitation.
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| Histologic features of FSGS? | LM shows segmental sclerosis, focal. IF: NO immune complex deposition… but may rarely see IgM in scarred areas. EM: extensive foot process effacement (>80%)
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| Amyloid path diagnosis? | AKI, CKD, + lambda light chains, +SPEP/EPEP, proteinuria, hyperglycemia, NODULES, linear staining on IF for mesangial collagen. congo red +apple-green birefringence
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| Renal microaneurysms are found in? | Polyarteritis nodosa
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| Polyarteritis nodosa affects which vessels? | small and medium sized arteries, especially Reno and visceral, nerves and coronaries… Causes aneurysms that look like tiny bead
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| Classic EM finding of Alports? | is variable thickening, thinning, basket weaving, and lamellation of GBM
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| Classic EM finding of thin basement membrane disease? | GBM Thickness measuring < 200nm
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| Dense deposit disease is best treated with _____. | eculizumab.
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| The best treatment for C3GN is —-. | Rituximab + plasma exchange
(eculizumab) has been shown to be useful in Rx of DDD, aHUS).
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| RITUXIMAB has been shown to be useful in RX of___. | ANCA vasculitis (induction or relapse per RAVE TRIAL), GPA(Wegener’s), HCV cryoglobulinemia or C3GN, TTP. [Rituximab has not been useful in DDD which is rx'd w ecu or plasma.]
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| Renal effects of antibodies against epidermal growth factor (cetuximab, panitumumab, matuzumab) | renal magnesium wasting in the distal tubule. Hypomagnesemia was associated with hypokalemia and hypocalcemia.
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| Syndromes that cause hypoMg | Barrters, Gitelmans
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