Glomerulonephritis Urine Microscopy Nephrotic Syndrome

Glomerulonephritis Urine Microscopy Nephrotic Syndrome
Nephritic Syndrome Rapidly Progressive Glomerulonephritis Cases Mark D. Purcell DO Nephrology/Internal Medicine Carolina Nephrology, PA 203 Mills Avenue Greenville, SC (864)

Pre-renal Post-renal Renal When someone’s GFR worsens 3 possibilities:
Think volume depleted (someone hasn’t been drinking enough)  decreased perfusion  GFR drops and creatine goes up Do not usually see a lot of etiologies in a UA Not many casts in the urine However, in prolonged prerenal acute renal injury  tubules sensitive  casts are produced (typically muddy, granular casts) Renal GN (glomerulonephritis) Postrenal Have something distal to the kidneys itself that causes dilatation of the urinary collecting system  hydronephrosis Happens from Stricture in ureters or something impinging on them or a stone in the bladder causing blockage Post-renal

Intrinsic (Renal) Causes of Acute Kidney Injury
Acute Glomerulonephritis (5%) Acute Tubular Necrosis Ischemic (50%) Toxins (35%) Acute Interstitial Nephritis (10%) Acute GN (AGN) only comprises only about 5% of clinical cases, maybe close to 10% ATN Ischemic develops with prolonged volume depletion When a pt is not perfusing their kidneys, cells along the tubules slough off  muddy granular casts in the urine Toxins, like Ab can cause this Aminoglygosides can do this AIN effects the interstitum of the kidneys ton of causes

Acute Glomerulonephritis 5-10% of ARF
Causes Anti-glomerular basement membrane antibody Disease Immune Complex Glomerulonephritis Pauci-immune Glomerulonephritis Diagnosis RBC cast Rapidly rising creatinine Renal biopsy findings Usually see some sort of immune complex attacking the glomerulus  Get RBC that creep into tubules  see RBC cast in the urine See rising creatinine from the dec GFR Need renal biopsy to accurately diagnose AGN

Essential in discussion of Glomerulonephritis (GN)
Urine Microscopy Looking at RBC and WBC that get through the glomerulus and into the tubules  get up into the matrix where tamm horsfall protein is secreted  Coats the cells  cells travels into urine and get plugged up into a cast The different kinds of casts help differentiate the causes of acute kidney injury Essential in discussion of Glomerulonephritis (GN)

Sedimentary Cells RBCs small, oval, clear WBCs
Leukocytes, which are larger PMNs Squamous epithelial cells much larger Think about size to help you differentiate the type of cast you are looking ffor

Epithelial Cell Cast Prominent nuclei
See the prominent nuclei due to the sloughing off of the epithelial cells Seen a lot in ATN (acute tubular necrosis) Tubules are either ischemic or toxins The prominent nuclei are what identify this as an epithelial cell cast Prominent nuclei

RBC Cast Long cylindrical cast
Small round clear oval cells = RBCs (black arrows) Pathologic for GN!!

Red Cell Cast 2 Urine of Acute GN

Red Cell Cast (broad)

Leukocyte Cast Bilobulated nuclei
Identified by PMNs or bilobulated nuclei Happens with AIN, lupus nephritis

Waxy Cast Characterized by the waxy character
See little indentations along the edge  signify heavy proteinuria or rapidly progressing GN

Granular & Hyaline Casts
Proteinatious material stuck in the casts will degrade over time  casts cannot move in the urine because so much tubular dysfunction = granular casts Most of us have hyaline casts, not pathologic Cleartamm horsfall protein in the matrix If had a kidney injury, usually see some other type of cast

Muddy Granular Casts ATN unless proven otherwise

Nephrotic Syndrome Protein gets into the tubules which tries to reabsorb some of it back into the body  get sloughing of tubular cells oval fat bodies Light microscopy (left) = oral, round, and bright Polarized light (right) = maltese cross This indicates heavy proteinuria Elsevier items and derived items © 2007 by Mosby, Inc., an affiliate of Elsevier Inc.

Urinalysis Your Analysis?
RBC cast  GN

Your Analysis? Muddy  ATN

Your Analysis? Leukocyte cast  AIN

Your Analysis?? Yeast (note budding and how much smaller it is to an RBC)

Case A 26-y/o male presents to your office as your first morning pt of the day. He c/o LE edema worse at the end of the day with “frothy urine – like the head of a beer.” He reports recent sinusitis for which he is using amoxicillin and OTC Ibuprofen. He denies any gross hematuria. PE is essentially normal except for significant peri-orbital edema noted. You perform a 24-hr urine and order some laboratories. Always ask if urine is bubbly or frothy - Albumin stabilizes bubbles so Protein in pee does this They swell up too - periprobital edema Esp after being asleep and waking up (laying down) and then swelling in legs at the end of the day CHF tend to have PND (proxisimial nocturnal dyspnea) and orthopnea (fluid collects in lungs) late at night Distinguish between this and swelling of legs from renal dysfunction Will always swell up if do not make enough protein  liver issues could be in DD as well but they tend to develop ascites rather than in legs or eyes - Ascities would make pt short winded bc pushing up on diaphragm, esp when they try to lay down at night

Case 24-hr urine reveals 16.8 gms protein with estimated creatinine clearance of 116 ml/min UA was negative for rbc’s BUN: 16.8 Cr: 0.8 Electrolytes: normal Complement levels: normal Really High protein in urine Normal creatinine clearance (another way we estimate GFR) BUN (Kidney function) correlates with the creatinine clearance

Case Which of the following is your NEXT step in the management of this patient? 1. Refer the patient for urgent renal biopsy 2. Start the patient on po prednisone given the proteinuria 3. Refer to a nephrologist 4. Have pt discontinue OTC agents 5. Have the patient perform an additional 24-hour urine b/c of your concerns of inaccurate collection It doesn’t resolve  2 This is happening because motrin - Have them stop NSAIDS

Acute GN

Definitions Nephrotic syndrome Nephritic syndrome
Massive proteinuria > 3.5 grams/day Hypoalbuminemia Hyperlipidemia Edema Non-inflammatory process Nephritic syndrome Hematuria and/or red cell casts Low grade proteinuria Hypertension Edema Decreased renal function Inflammatory process Nephrotic Liver tries to make up for loss of protein, so ends up making more lipids too Edema because protein is gone and albumin cannot hold protein where it needs to be Nephritic Much more inflammatory process Tend to have HTN vs nephrotic pts do not Edema is similar between the two In general, it tends to cause more rapid loss of renal function

Glomerulonephritis Acute renal failure associated with hypertension, hematuria, and edema Decreased urine output with azotemia Result of inflammatory attack on glomerulus Can be primary kidney disorder or secondary to another systemic disease Hallmark is hematuria and red cell casts azotemia Increased nitrogenous waste products in the body  measured by BUN levels (very high) Smokey, tea/cola colored urine

Causes of Glomerulonephritis
Primary Renal Disorders Secondary Renal Disorders Membranoproliferative GN Cresentric GN Fibrillary GN IgA nephropathy SLE Post-streptococcal GN Hepatitis C Vasculitis-related Wegener’s Granulomatosis Microscopic Polyangiitis Churg-Strauss Syndrome Infective Endocarditis

Glomerular Filtration Barrier
Geometry GVEC block 90% of total SA Fenestrated endothelium Bottom of image = blood coming into the capillaries of the glomerulus RBCs and albumin should stay here if have good functioning GMB because of the following: There are neg charged proteoglycans in endothelial cells and neg charged proteoglycans (heparins) in GBM Albumin is negative so they repel - Even if cells get across this, there are GBM = glomerulus basement membrane Have foot processes produced by podocytes Have slit diaphragms between these Copyright ©2007 American Society for Clinical Investigation Quaggin, S. E. J. Clin. Invest. 2007;117: 27

Nephrotic Syndrome ≥ 3.5 gm/d
Frequently associated with underlying glomerular disease Hypoalbuminemia, hyperlipidemia, and edema

Nephrotic Syndrome Minimal Change Disease
Focal Segmental Glomerulosclerosis (FSGS) Membranous Diabetic Nephropathy Most common cause of nephrotic syndrome in the US Amyloidosis * MPGN (usually 2º cause) Membranal proliferative GN  not liking this Can only cause nephrotic syndrome if it is due to something else In general when you see this think nephritic syndrome

Minimal Change Disease
Most common disease in children. Benign urinary sediment, normotensive, preserved renal function 85% responsive to steroids Secondary causes include NSAIDS, Non-Hodgkin’s Lymphoma Liquid tumors Abrupt onset (like the original case) >8-10g protein in urine (profound proteinuria) Do not have a lot of RBC casts or anything like that on UA Most common cause of GN in young adults (up to age 25) *Liquid tumors

Normal Glomerulus on LM Minimal Change Disease
Open capillary loops like normal Only 1 – 2 cells in the capillary tuft like normal Wall of the capillary loop is essentially the same thickness as the tubule walls (normal) Looks normal under light microscopy Courtesy of

Minimal Change Disease
Electron microscopy Podocytes are fused (afaced) Only change we see (cannot make out slit diaphragms) Most cases are responsive to stopping the offending agents and then if that doesn’t work start steroids (if no secondary cause) Elsevier items and derived items © 2007 by Mosby, Inc., an affiliate of Elsevier Inc.

Focal Segmental Glomerulosclerosis
Primary or secondary May present w/ renal insufficiency, HTN in addition to nephrotic syndrome Multiple secondary causes May respond to immunosuppression Collapsing glomerulopathy Worst variant 2 H’s and 2 P’s Inevitable deterioration to ESRD Epidemic in African Americans 2H’s and 2P’s of collapsing variant: HIV, heroine nephropathy, parvo virus, pamidronate KNOW THE H’s! ESDR – end stage renal disease

HIV Nephropathy (HIVAN)
Collapsing FSGS Echogenic/Large Kidneys Almost exclusively in African Americans with profound proteinuria Treatment is HAART, ACE Inhibitors, corticosteroids (controversial), dialysis 9 ½ – 11 ½ cm = normal kidney size, and these pts have 13 – 14 cm Major proteinuria (9-10g) but only minimal edema * One of the few conditions where we see this presentation

Focal Segmental Glomerulosclersosis
Primary/Idiopathic: Caused by circulating permeability factor Secondary Causes: Viral (HIV) Obesity Hyperfiltration injury Prior nephrectomy sickle cell disease reflux nephropathy IV drugs (“Heroin nephropathy”) reflux nephropathy – pee refluxes back to the kidney Can develop FSGS as adults b/c bad kidney that had the reflux likely will be removed as a child. Either way, the good kidney has to enlarge to compensate for a bad or missing kidney  FSGS can develop

Membranous Glomerulopathy
Primary or secondary Associated with malignancy in elderly Spontaneous resolution in 1/3 of patients High risk groups may respond chemotherapy PE/Renal Vein Thrombosis Don’t screw up with membranal proliferative GN (mpgn more nephritic and this is more nephrotic) Do cancer screens in these pts (Esp if older) If smoker, get a chest X-ray If haven’t had exams such as colonoscopy get that done Remember to watch out for PE/Renal Vein Thrombosis if you have a membranous glomerulopathy pt come in with flank pain or dyspnea This has the highest risk out of all the kidney screw ups for PE/Renal Vein Thrombosis

Membranous Nephropathy: Secondary Causes
Infection: Syphilis, Hepatitis B, HIV Medications: NSAIDs, Captopril Malignancy: solid tumors SLE Note solid tumors  membranous nephropathy Liquid tumors  minimal change nephropathy Note how NSAIDs cause both this and minimal change  need to do biopsy to actually confirm Note how HIV can cause both this and FSGS  need to do biopsy to actually confirm

Class I: Minimal mesangial lupus nephritis
Abbreviated International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis (2003) Class I: Minimal mesangial lupus nephritis Class II: Mesangial proliferative lupus nephritis Class III: Focal lupus nephritisa Class IV: Diffuse segmental (IV-S) or global (IV-G) lupus nephritisb Class V: Membranous lupus nephritisc Class VI: Advanced sclerosing lupus nephritis Not responsible for this a Indicate the proportion of glomeruli with active and with sclerotic lesions. b Indicate the proportion of glomeruli with fibrinoid necrosis and cellular crescents. c Class V may occur in combination with class III or IV in which case both will be diagnosed. Indicate and grade (mild, moderate, severe) tubular atrophy, interstitial inflammation and fibrosis, severity of arteriosclerosis or other vascular lesions.

Systemic lupus erythematosus
Subendothelial deposits Note see subendothelial deposits with lupus  membranous nephropathy can occur

Membranous Nephropathy
Can see deposits Thickened basemen membranes* - This is what you tend to see with membranous GN Elsevier items and derived items © 2007 by Mosby, Inc., an affiliate of Elsevier Inc.

Diabetes Mellitus Early Stages of Diabetic Nephropathy
Most common cause of nephrotic syndrome in the US, but not world wide* See nodular glomerular sclerosis (yellow circles top left) Aka kimmelstien wilson nodules DO NOT biopsy a pt with DM nehrophathy bc it doesn’t chance the course of tx. Start with developing proteinuria, so look for that on a dip stick with a UA The pt will get more and more protein in the urine over the years If the pt gets DM retinopathy then prone to getting DM nephropathy Tx = strict glucose control and ACEs or ARBs Example: Lets say you have a 37 yo pts diagnosed with DM two year ago, and comes in with acute onset of leg swelling this is NOT DM nephropathy This would be too short of a timeline to be DM nephropathy. This takes years to develop (10 – 20 years before we’d see this in a pt) This would be more of an acute onset in this scenario Late Stages of Diabetic Nephropathy

Amyloidosis Congo Red positive staining eosinophilic, amorphous deposits w/in kidney AL – associated w. Lamda light chains lambda “lays down amyloid” kappa “kills”, light chain nephropathy Negative Congo red staining Immunotactoid Fibrillary GN Two types of myeloma Kappa and lambda Need to prove by biopsy (and diagnose by S-peps and U-peps (?) ) Abdominal fat pad or Kidney Lambda lays down the amyloid and it gets into the kidneys  cannot be reabsorbed  overall, high deg or proteinuria Kappa  kills the glomerulus  more acute kidney injury  dec GFR  inc creatinine Don’t worry about the Immunotactoid or Fibrillary GN

END OF LECTURE 5 Begin Lecture 6
Begin discussion of Nephritic End Nephrotic

RBC Cast Round, thin RBC membranes
Classic for GN, esp nephritic syndrome

Dysmorphic Glomerular Erythrocytes
Elsevier items and derived items © 2007 by Mosby, Inc., an affiliate of Elsevier Inc. If you not get a RBC cast, can get dysmorphic RBCs Look like “mickey mouse cells” with little ears coming off of the ovals Dysmorphic  not uniform in the shape of regular RBCs It is thought that as the RBCs get from the blood, pass through the GBM and podocyte foot processes  RBCs get deformed  travels throught the tubules with all these physical and chemical insults = dysmorphic RBCs Know this, or a RBC cast indicates GN

Case Vignette #2 A 15 yo M c/o dark colored urine. He is found to have 3+ proteinuria, numerous RBC’s, RBC casts. Scr = 2.4. What is your first question? Family history? Any medication recently? When was your sore throat? Have you been drinking excessive cola drinks? Scr of 4 – 5 coorelates with a GFR of about <10 – 15….renal dialysis No one gives a shit about drinking coke…seriously….

Immune Complex GN Timing of events C3, C4 low; ASO titres
Antinuclear Antibody Therapy No biopsy needed for post-infectious GN No treatment necessary for post-infectious GN ASO – indicates strep throat Only + in about 75% of cases so do not rule it out if the titer is neg ANA – test for SLE(which can cause nephritic and nephrotic GN)

Post-streptococcal GN
Nephritic syndrome Have increased PMNs and monocytes See a lot of activity in the glomerulus if did a kidney biopsy

Post-streptococcal GN
With post strep GN see subEPIthelial (versus subENDOthelial which you see with SLE nephrotic syndrome (note he said nephritic…but pretty sure he screwed it up…) ) Sub epithelial humps

Acute Post-Streptococcal Glomerulonephritis
Latent period 6-10 days after throat infection 10-14 days after cutaneous infection Nephritogenic steptococi identified by serotyping a cell wall antigen (M protein) Immunity to M protein is type specific & long lasting Common in children 6-10 years Pathology: diffuse endocapillary proliferative GN with PMN and Monocyte Infiltration and crescents I.F. granular IgG and C3, EM: subepithelial humps Note pt will have tea colored urine usually Timing AFTER the throat issue is impt (bc there is another kidney issue that goes concurrently with the sore throat) Immune complex deposits cause low complement levels Have a low C3 that is SHORT LASTING in Post strep GN (usually <3mo) So note that if a pt comes in complaining of issues like this with the kidneys, tells you that they were tx for un URI about 3 mo ago, their C3 is still low, it is prob not post strep GN and other type of GN

NEPHRITIC SYNDROME Oliguria Hematuria with red cell casts
Proteinuria - usually < 3 gms/day Edema HTN abrupt onset Things that happen in general Oliguria The amount of urine you make a day (< 400ml a day) FYI less than 100ml a day = anuria >400ml a day non-oliguria (usually the case in nephrotic) Proteinuria is usually SUBnephrotic - Nephrotic would be >3 or 3.5 g of protein in the urine (see with nephrotic syndrome)

Nephritic SLE Post-Infectious GN (PIGN) IgA Nephropathy (Berger’s Dz)
HSP Membranoproliferative Glomerulonephritis (MPGN) IgA Nephropathy (Berger’s Dz) The most common GN in the world (remember DM nephropathy is the most common in the US) Do a kidney biopsy to truly diagnose one of these Can get clues to what is going on by the timing of the hematuria (like with post strep GN) and complement levels

IgA Nephropathy Most common glomerular disease worldwide*
Episodic hematuria Acute with infection and exercise Majority benign: 85-90% renal survival at 10 yrs. Treatment ? Fish Oil Anti-RAAS agents Prednisone/Immunosuppressants Normal complements/C3 levels Pt starts with pharyngitis the night before, will come to see you the next day and let you know they have a cough and their urine is tea colored = acute hematuria WITH the infection, versus 6-10 days afterwards (like it was with post strep GN) Also, if pt undergoes rigorous activity or exercises they may develop gross hematuria Most cases are benign Fish oil is a debatable tx If they have HTN  use ACEI or ARBs If they have a flare up or rapid progression in a decrease in their GFR then treat with steroids or immunosuppressant Normal complement levels impt (where it is low in post strep for about 3mo or less) Henoch–Schönlein purpura - variant of the disease, more common in kids  vasculitis issues

Membranoproliferative Glomerulonephritis (MPGN)
SLE Hepatitis C ↓ C3 level Do hep workups on these pts Esp if they have had a transfusion Pts will have a persistent low C3 level that happens greater than 3 mo

Membranoproliferative Glomerulonephritis (MPGN)
Type I: Mesangiocapillary GN Type II: Dense deposit Type III: Mixed Type I, membranous features Secondary causes: Hepatitis C Autoimmune disease Dysproteinemias Not really responsible for knowing the different types, just know any kind of membranopiliferative shit causes GN KNOW Hep B with membranous GN (which is usually nephotic) whereas Hep C causes MPGN (usually nephritic)

Clues to Cryoglobulinemia
Hepatitis C positive Palpable purpura (leucocytoclastic vasculitis), livedo reticularis, leg ulcers, acrocyanosis, Raynaud’s phenomenon, necrosis of tips of digits Arthralgias, weakness Hepatosplenomegaly or LFT abnormalities Peripheral neuropathy (mononeuritis multiplex), CNS or abdominal vasculitis, pulmonary hemorrhage Markedly low C4, normal or slightly depressed C3 Strongly positive Rheumatoid Factor Usually severe leg rash (but it can be anywhere in their body) Multisystem disease Joint problems Liver abnormalities When this effects the kidneys = GN Note complement level

Hypocomplementemia in Renal Disease
SLE nephritis: C3 and C4 equally depressed PSGN (PIGN): Low C3, Normal C4 Lasts < 3 months MPGN: Low C3, normal C4 Lasts > 3 months (persistent) Cryoglobulinemic GN: Markedly low C4, normal or slightly depressed C3 Miscellaneous: Atheroembolism, HUS/TTP, liver disease, and sepsis (decreased synthesis) All of these are immune complex issues Autoimmunity issues where the body cannot produce enough complement to fix the issue quickly  destruction issue

Vasculitis

Vasculitis Multi-Organ involvement Mononeuritis multiplex
Nerve areas in two separate parts of the body are affected Classify according to vessel size** Muscle ache, skin pain, abdominal pain, skin rash, etc… = vasculitis We will discuss small vessel vasculitis (effects those vessels smaller than arteries) Pt will come in with complaints, such as blood in urine and kidney dysfunction + hemoptysis because of lung involvement that can occur Prob don’t have to know this but…: In general, medium vasculitis effects arteries kawasaki vasculitis  effects kids polyarteritis nodosa effects the renal a, so pts come in with flank pain from destruction and likely hematuria Large vessel vasculitis Temporal vasculitis takayasu vasculitis

Systemic Vasculitis Know small vessel vasculitis effects vessels smaller than arteries

Small Vessel Vasculitis
Pauci-Immune Wegener’s Granulomatosis Churg-Strauss Syndrome Microscopic Polyangiitis Immune-Complex Henoch-Schonlein Purpura Essential Cryoglobulinemic Vasculitis Henoch-Schonlein Purpura A small subset of IgA nephropathy Usually involves the skin, kidney, and gut Involves abdominal pain, skin vasculitis for the most part, but can involve kidney Essential Cryoglobulinemic Vasculitis - The same thing as Cryoglobulinemic GN, but the vasculitis form just effects the skin more

Anti neutrophilic cytoplasmic antibodies (ANCA)
c-ANCA p-ANCA This suggest a pauci – immune condition Measure in the urine Proteinase-3 Myeloperoxidase

Approximate Frequencies of ANCA’s in Pauci-Immune Vasculitis
Microscopic Polyangiitis Wegener’s Granulomatosis Churg-Strauss Syndrome c-ANCA (PR3) 40% 75% 10% p-ANCA (MPO) 50% 20% 60% ANCA negative 5% 30% Know what is bold

Wegener’s Granulomatosis
Necrotizing granulomatous inflammation Respiratory tract involvement Pt will be pretty sick Hemoptysis Acute kidney injury Usually URI the past wk or so  cough up blood  necrotizing granulomatous inflammation PT WILL HAVE A BONE DEFORMITY ON THEIR NOSE – Saddle nose Hoarseness (involvement of trachea) Hearing loss This almost always involves the lungs and have a nose deformity

Treatment of Wegener’s granulomatosis
Prompt recognition IV methylprednisolone Cyclophosphamide Trimethoprim-sulfamethoxasole Intravenous immunoglobulin (Plasmapheresis) Use IV steroids, 1g a day for about 3 days until biopsy can be done If results come back + for wegners  give immunosupressant IVIG or plasmapheresis is a possibility depending on the amount of kidney injury they have

Cyclophosphamide Short term side effects Long term side effects
alopecia neutropenia nausea/emesis opportunistic infection hemorrhagic cystitis Long term side effects Malignancy amenorrhea / sterility Know SE Pts can have significant hematuria, it can actually back up because clots can get so big Using too much immunosuppressant can cause opportunistic infections = hematuria Goal for diagnosis of hemorrhagic cystitis is cystoscopy Caution with young females and SLE  sterility

Churg-Strauss Syndrome
Necrotizing granulomatous inflammation Asthma Eosinophilia Renal involvement less common than Wegener’s and Microscopic Polyangiitis Rrreeeaaaaalllllllyyyy high eosinophilia Biggest complaint is peripheral neuropathy and possibly some heart block

Microscopic Polyangiitis
Absence of granulomatous inflammation Absence of asthma and eosinophilia p-ANCA positive Differentiate from Polyarteritis nodosa (P-AN) Know what microscopic – capillary involvement and/or P-ANCA (not with PAN)

Case Vignette #3 The most likely serum findings?
A 44 yo F has generalized arthralgias, chronic sinusitis of 6 months duration, experiences hemoptysis with cough and R foot drop. PE: BP 150/94 Afebrile Saddle nose deformity, no rash or joint effusions, R basilar ronchi. 1+ pitting edema, strength 3/5 in dorsiflexion of R foot. CXR reveals bilateral infiltrates, Scr 3.2 with active urinary sediment. Active urinary sediment  A – SLE B – Wegners The most likely serum findings? Hypocomplementemia, ANA +, dsDNA + + C-ANCA (anti-proteinase3) + P-ANCA (anti Myeloperoxidase) Hepatitis C Antibody +, complement normal

Rapidly Progressive Glomerulonephritis (RPGN)
Rapidly rising Scr ( i.e. acute renal failure) Active urinary sediment (RBC casts) Nephritic proteinuria (<3.5 gm / 24hrs.) Crescentic Glomerulonephritis Rapid recognition, therapy essential to preservation of renal function Nephrology emergency Kidneys will shut down in a day – a week If have pulmonary involvement where they are bleeding in their lungs they can die quickly Know involves kidneys + lungs You would need to do a biopsy to diagnose this, but you have to move really, really fast to save the pt. The 1st thing you should do is IV steroids - If have really high suspicions, can start on immunosuppressant

Rapidly Progressive GN
Anti-GBM Goodpasture’s Disease Immune Complex PIGN SLE Pauci-immune (ANCA GN) Wegener’s GN Microscopic PAN Endocarditis Note crescent shape = Crescentic Glomerulonephritis Bad diagnosis to have An anti- GBM disease that involves  Goodpasture’s disease

Pulmonary – Renal Syndrome
More Common Goodpasture’s Disease SLE Microscopic Polyangiitis Wegener’s Granulomatosis Less Common Churg-Strauss Syndrome Henoch-Schonlein purpura Hemolytic uremic syndrome Behcet’s disease Mixed cryoglobulinemia Rheumatoid vasculitis Penicillamine treatment Means effects the lung and kidney (gee really…..never would have guessed that) - This is not good  means acute kidney dysfunction and bleeding in the lungs Elsevier items and derived items © 2007 by Mosby, Inc., an affiliate of Elsevier Inc.

Case Vignette #4 A 64 yo previously healthy WF arrives to ER c/o dyspnea, hemoptysis, bilateral alveolar infiltrates, and serum creatinine of 3.6 mg/dL. UA reveals 3+ protein, numerous RBC’s, RBC casts. What is your next appropriate step? CT scan of chest r/o pulmonary embolism Kidney biopsy Bronchoscopy w. alveolar washing and brush biopsy Bolus IV Methylprednisolone (1 mg/kg) daily for 3 days Start on steriods and then do the kidney biopsy Watch out for CT scan with contrast if have screwed up kidneys

Electron Microscopy Immunofluorescence
Little immune bodies against the GBM Linear staining pattern for antiGBM antibodies

Diagnostic Evaluation
Prompt recognition Bi Modal Age distribution: :20-30:60-70 High correlation smoking, hydrocarbon exposure Anti GBM antibodies ANA, C3, C4 Anti-Neutrophilic Cytoplasmic Antibody titer DLCO Renal Biopsy

Treatment for Anti-GBM Disease
Prompt recognition IV Methylprednisolone Plasmapheresis Cyclophosphamide

Cyclophosphamide Remission rates 93% Short term side effects
alopecia neutropenia nausea/emesis opportunistic infection hemorrhagic cystitis Long term side effects Malignancy amenorrhea / sterility THIS IS FOR WEGNERS, NOT ANTI-GBM - Remission rates 93% Know SEs

Treatment of GN General Measures Anti-HTN agents Diuretics for edema
Hemodialysis for kidney failure Steroids for systemic disease Plasmapheresis for immune complex disease Antibiotics for infectious causes

Treatment of GN Supportive treatment of HTN, proteinuria, edema, hyperlipidemia, hypercoagulability Methylprednisolone Cyclophosphamide Mycophenolate Mofetil Imuran Cyclosporine, Prograf Rituximab Plasmapharesis ASA, Persantin, coumadin

Case A A 19-year-old woman is evaluated for sudden-onset periorbital and pretibial edema. Three weeks ago, she was diagnosed with pharyngitis that has since resolved without treatment. On physical examination, blood pressure is 150/100 mm Hg. Cardiac examination reveals a normal S1 and S2 with a soft S3. The lungs are clear and the abdominal examination is normal. There is periorbital and bilateral pitting pretibial edema. There are no rashes.

Laboratory Studies Blood urea nitrogen 30 mg/dL Creatinine 1.5 mg/dL
Albumin 3.8 g/dL Complement (C3) 15 mg/dL (low) Complement (C4) 48 mg/dL (normal) Urinalysis: 1+ protein; several erythrocytes, dysmorphic erythrocytes, and erythrocyte casts/hpf

Which of the following is the most likely diagnosis?
A. Antineutrophil cytoplasmic antibody–associated small-vessel vasculitis B. Goodpasture's syndrome C. IgA glomerulonephritis D. Postinfectious glomerulonephritis E. Systemic lupus erythematosus nephritis

Case B A 21-year-old woman is evaluated in the office for facial and lower-extremity edema of 1 week's duration. For the past 3 weeks, she has had fatigue. She has no history of diabetes mellitus, cigarette smoking, or illicit drug use. On physical examination, blood pressure is 90/55 mm Hg. Cardiac and pulmonary examinations are normal. The abdomen is soft and without masses. There is periorbital edema and 2+ lower-extremity edema.

Laboratory Studies Creatinine 0.7 mg/dL Total cholesterol 325 mg/dL
Albumin 2.9 g/dL Complement (C3 and C4) Normal Urinalysis Specific gravity 1.026, 3+ protein 24-Hour urine protein excretion 15 g/24 h

Profound proteinuria

Which of the following is the most likely diagnosis?
A. Focal segmental glomerulosclerosis B. Membranoproliferative glomerulonephritis C. Membranous nephropathy D. Minimal change glomerulopathy E. Systemic lupus erythematosus nephritis Abrupt onset, profound proteinuria

Mark D. Purcell, DO e-mail: mpurcell@mykidneydoc.com
Questions Mark D. Purcell, DO

Glomerulonephritis Urine Microscopy Nephrotic Syndrome

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