unusual cause of renal failure

57
Physicians Meet – M2 Case Presentation Prof. Dr. S. Sundar’s Unit Presented by Dr. Deepu Sebin

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Page 1: Unusual cause of Renal failure

Physicians Meet – M2Case Presentation

Prof. Dr. S. Sundar’s Unit

Presented by Dr. Deepu Sebin

Page 2: Unusual cause of Renal failure

Ponnammal , 55 yr old female House wife First admitted with complaints of

Abdominal distention – 1 month Swelling of legs – 1 month

Page 3: Unusual cause of Renal failure

Gradually progressing abdominal distention over one month.

Breathlessness with moderate exertion. No orthopnea / PND. Sweling of both legs for 1 month Swelling of legs mainly in the evenings. No history of nausea or vomiting. No history of malena/hematemesis. No history of decreased urine output. No history arthralgia/ oral ulcers

Page 4: Unusual cause of Renal failure

Past History No history DM/SHT/PTB/ Jaundice/ CAD/

Surgeries/Drug Intake No history of blood transfusions

Personal History Post Menopausal status Mixed Diet

Family History Nil Specific

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General Examination PR – 80/min BP - 110/70 mmHg JVP – elevated No pallor/cyaosis/clubbing/lymphadenopathy BPPE + No Signs of liver cell failure

Page 6: Unusual cause of Renal failure

Systems P/A

Distented Free Fluid + No organomegaly BS +

Chest Clear AEBE

CVS S1 S2 normal

CNS NFND PEARL

Page 7: Unusual cause of Renal failure

Investigations Hb - 10 mg/dl TC – 5600 DC – P60 L30 ESR – 10mm/hr Platelet Count – 1.4 lakh

RBS – 128 mg/dl Blood Urea – 42 mg/dl S. Creatine – 1.2 mg/dl

Na+ - 139 K+ - 3.7

Page 8: Unusual cause of Renal failure

Urine Rountine Albumin - + Sugar – Nil Depostis – 1 -4 pus cells

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Bilirubin – 1.0 mg/d SGOT- 83u/l SGPT – 49u/l ALP – 146u/l Total Protein – 6.1 g.dl Albumin – 4.0 g/dl Globulin – 1.8 g/dl

INR 1.2

Page 10: Unusual cause of Renal failure

USG Abdomen : Liver 10.1 cm shrunken, coarse echo texture,

surface nodular. GB wall thickened Spleen 13 cm enlarged, no focal lesions Kidneys

Rt 8.8 x 4.3 Lt 8.4 x 4.0 Normal echotexture, CMD maintained

Bladder normal Impression : Cirrhosis with Portal Hypertension

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HbSAg – positive Anti HCV – negative HIV – negative OGDscopy – Grade 2 fundal varices

Page 12: Unusual cause of Renal failure

Impression : DCLD with Portal Hypertension HBV Related

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2nd admission Patient got readmitted with increasing

abdominal distention and breathlessness of 1 wk duration.

Vitals Stable General Examination – Facial puffiness

BPPE + Systems P/A

Distended Free fluid No organomegaly

Page 14: Unusual cause of Renal failure

Hb- 11mg/dl TC – 6400 DC – P60 L30 ESR – 10mm/hr Platelet Count – 1.1lakh

RBS – 111 mg/dl

Blood Urea – 67 mg/dl S. Creatine – 1.8 mg/dl

Na+ - 136 K+ - 3.7

Page 15: Unusual cause of Renal failure

Bilirubin – 1.0 mg/d SGOT- 90 u/l SGPT – 53 u/l ALP – 145 u/l

Total Protein – 5.9g.dl Albumin –2.9 g/dl Globulin – 1.8 g/dl

INR 1.2

Page 16: Unusual cause of Renal failure

Urine Rountine Albumin - + Sugar – Nil Depostis –1-3 pus cells 10-12 RBCs

Page 17: Unusual cause of Renal failure

DCLD – Portal Hypertension , HBV related AKI - ? Cause to r/o HRS Non oliguric

Page 18: Unusual cause of Renal failure

Urine Sodium – 22meq (<20 pre renal & >40 ATN)

                                   UNa   x   PCr          FENa, percent    =       — — — — — —x

100 —                                               PNa   x   UCr FeNa – 0.8 (<1 prerenal , > 2 ATN )

Page 19: Unusual cause of Renal failure

Plasma Urea / Creatine Ratio – 37.2 (>30 prerenal)

Urine to plasma creatine ratio - 24 (<20 ATN , >40 pre renal )

Urine Creatininre – 43mg/dl

Page 20: Unusual cause of Renal failure

Diuretics including Spironolactone stopped Syrup Laculose temporarly withdrawn Antibiotics ( Cefotaxime, Metrogyl ) initiated Maintained adequate fluid intake orally Hypovolemia ruled out

No hematemesis or malena No loose stools

Page 21: Unusual cause of Renal failure

1st adm

Day 14

Day15 Day 18

Day 20

Day 22

Day 28

Day 29

Blood Urea

42 67 56 60 49 56 63 59

S.Creatinine

1.2 1.8 1.7 1.6 1.4 1.8 1.7 1.8

Urine Albumin

+ Nil ++ ++ +++ +++ ++ +++

24 hour urine protein – 2gm Volume 1500 ml

Page 22: Unusual cause of Renal failure

In view of the Elevated renal parameters Which is static Not resolving with conservative management Proteinuria

Proceeded with Renal Biopsy after Nephrology Review

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Sections show renal tissue with eight glomeruli per sections. These are enlarged and hypercellular with endothelial and mesangial proliferation with infiltration by few neutrophils. Focal splitting of capillary wall noted. No capillary wall thickening is seen. There is no tubular atropy. Blood vessels appear unremarkable

Immunofluorescence stains show peripheral granular deposits in IgM, IgG, IgA, C3c, and C1q

Impression : Renal Biopsy showing Diffuse Proliferative Glomerulonephritis and mild exudation and no significant tubuointerstitial changes.

Page 26: Unusual cause of Renal failure

Full House pattern IgM, IgG, IgA, C3c, and C1q

Possibility of Lupus Nephritis, vasculitis and other connective tissue disorders with DPGN – full house pattern.

Page 27: Unusual cause of Renal failure

ANA – negative (Repeated) ds DNA – negative ASO titer - < 100 p-ANCA and c- ANCA – Negative

C3 – 53.04 (90 – 180mg/dl) C4 – 13. 78 (10- 40 mg/dl)

Page 28: Unusual cause of Renal failure

HBsAg – Postive HBeAg – Positive HBV DNA levels – > 20,000 IU/mL

Page 29: Unusual cause of Renal failure

In Hepatits B virus related Glomerular pathology we expect membranous glomerulonephritis, or

membranoproliferative glomerulonephritis

Here we have Active Hepatitis HbeAg postivity High Viral Load

Absence of other infection, Connetive tissue disorders clinically and radiologicaly.

This is a case of HBV related DPGN

Page 30: Unusual cause of Renal failure

Final Diagnosis HBV infection related Diffuse Proliferative

Glomerulonepheritis HBV related DCLD and PHT

Am J Gastroenterol. 1995 Jun;63(6):476-80.Hepatitis-B-antigenemia with panarteritis, diffuse proliferative glomerulitis and malignant hypertension.Razzak IA, Bauer W, Itzel W.

Full-house nephropathy in a patient with negative serology for lupus Esra Baskin, Pınar Isik Agras, Nurcan Menekşe, Handan Ozdemir and Nurcan Cengiz

Page 31: Unusual cause of Renal failure

Treated with T. Lasix 40mg BD T. Lamividine 100mg OD T. Propranalol 20 BD T. Rantac 50mg BD Syp. Lactulose 15ml TID Pulse Methylprednisolone therapy reserved if

patient develops worsening of her renal failure.

Page 32: Unusual cause of Renal failure

Simplified Appraoch to Glomerular Disease

Glomerular Disease a diagnosis before biopsy

— Although there are many causes of glomerular disease, the patient's age and

the characteristics of the urine sediment usually allow the differential

diagnosis to be narrowed prior to renal biopsy

Page 33: Unusual cause of Renal failure

Focal Nephritic

Diffuse Nephritic

Nephrotic

Page 34: Unusual cause of Renal failure

Focal nephritic  Focal nephritic — 

Focal glomerulonephritis is associated with inflammatory lesions in less than one-half of glomeruli on light microscopy.

The urinalysis reveals red cells (which often have a dysmorphic appearance) red cell casts, and mild proteinuria (usually less than 1.5 g/day).

The findings of more severe disease are usually absent, including nephrotic range proteinuria, edema, hypertension, and renal insufficiency. These patients often present with asymptomatic hematuria and proteinuria discovered on routine examination.

Page 35: Unusual cause of Renal failure

Focal Nephritic - DDs Active urine sediment without renal insufficiency or

nephrotic syndrome

Less than 15 years - Mild postinfectious glomerulonephritis, IgA nephropathy, thin basement membrane disease, hereditary nephritis, Henoch-Schönlein purpura, mesangial proliferative glomerulonephritis

15 to 40 years - IgA nephropathy, thin basement membrane disease, lupus, hereditary nephritis, mesangial proliferative glomerulonephritis

Greater than 40 years - IgA nephropathy

Page 36: Unusual cause of Renal failure

Diffuse Nephritic Diffuse nephritic —

 Diffuse glomerulonephritis, in comparison, affects most or all of the glomeruli.

Thus, the urinalysis is similar to focal disease, but heavy proteinuria (which may be in the nephrotic range), edema, hypertension, and/or renal insufficiency may be seen.

Page 37: Unusual cause of Renal failure

Diffuse Nephritic - DDs Active urine sediment with renal insufficiency and

variable proteinuria, which can include nephrotic syndrome Less than 15 years - Postinfectious glomerulonephritis,

membranoproliferative glomerulonephritis

15 to 40 years - Postinfectious glomerulonephritis, lupus, rapidly progressive glomerulonephritis, fibrillary glomerulonephritis, membranoproliferative glomerulonephritis

Greater than 40 years - Rapidly progressive glomerulonephritis, vasculitis (including mixed cryoglobulinemia), fibrillary glomerulonephritis, diffuse proliferaive nephritis, postinfectious glomerulonephritis

Page 38: Unusual cause of Renal failure

Nephrotic —  Heavy proteinuria and lipiduria, But few cells or casts Bland proteinuria Patients who also have edema and hyperlipidemia (the

full-blown nephrotic syndrome) tend to have a more marked glomerular leak than those with heavy proteinuria alone.

Differntial Diagnosis – all the range of nephrotic disorders

Patients with Nephrotic proteinuria but no hypoalbuminemia or edema – Suspect some form of secondary focal glomerulosclerosis (as with reflux nephropathy)

Page 39: Unusual cause of Renal failure

The relatively bland sediment in the nephrotic disorders results from the lack of inflammatory cell infiltration in the

glomeruli absence of immune complex deposition in most of

these disorders

The lack of inflammation also results in the plasma creatinine concentration being normal or only slightly elevated at presentation in the nephrotic disorders !

Page 40: Unusual cause of Renal failure

Renal Failure + Nephrotic Syndrome Concurrent acute tubular necrosis, esp in MCD Tubular injury in collapsing focal

glomerulosclerosis, either idiopathic or associated with HIV infection. (Collapsing FSG)

Minimal change disease with acute interstitial nephritis due to NSAIDs

Crescentic glomerulonephritis superimposed upon membranous nephropathy.

Nephrotic syndrome secondary to monoclonal immunoglobulin deposition disease may also develop myeloma cast nephropathy and acute renal failure.

Page 41: Unusual cause of Renal failure

Nephrotic - DDs Heavy proteinuria, bland sediment although some

hematuria allowed . Less than 15 years - Minimal change disease, focal

glomerulosclerosis, mesangial proliferative glomerulonephritis

15 to 40 years - Focal glomerulosclerosis, minimal change disease, membranous nephropathy (including lupus), diabetic nephropathy, preeclampsia, postinfectious glomerulonephritis (later stage)

Greater than 40 years - Focal glomerulosclerosis, membranous nephropathy, diabetic nephropathy, minimal change disease, IgA nephropathy, primary amyloidosis or the related disorder light chain deposition, benign nephrosclerosis, postinfectious glomerulonephritis (later stage)

Page 42: Unusual cause of Renal failure

References Comprehensive Clinical Nephrology 3rd edition Rose, BD. Pathophysiology of Renal Disease, 2d ed, McGraw-Hill,

New York, 1987, p. 16

Rose, BD. Clinical characteristics of glomerular disease. In: Scientific American Medicine, Rubinstein, E, Federman DD (Eds), Scientific American, New York, 1989, section X (IV):1. Praga, M, Borstein, B, Andres, A, et al. Nephrotic proteinuria without hypoalbuminemia:

Clinical characteristics and response to angiotensin-converting enzyme inhibition. Am J Kidney Dis 1991; 17:330.\

Rivera, F, Lopez-Gomez, JM, Perez-Garcia, R, et al. Clinicopathologic correlations of renal pathology in Spain. Kidney Int 2004; 66:898. Iseki, K, Miyasato, F, Urhara, H, et al.

Outcome study of renal biopsy patients in Okinawa, Japan. Kidney Int 2004; 66:914

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Renal Failure in Cirrhosis Hepatorenal Syndrome Psuedohepaorenal Syndrome

Page 44: Unusual cause of Renal failure

HRS – DIAGNOSIS Major Criteria — :

Chronic or acute hepatic disease with advanced hepatic failure and portal hypertension

A plasma creatinine concentration above 1.5 mg/dL that progresses over days to weeks.

The absence of any other apparent cause for the renal disease, including shock, ongoing bacterial infection, current or recent treatment with nephrotoxic drugs,

The absence of ultrasonographic evidence of obstruction or parenchymal renal disease.

Urine red cell excretion of less than 50 cells per high power field (when no urinary catheter is in place)

Protein excretion less than 500 mg/day. Lack of improvement in renal function after volume expansion with intravenous

albumin (1 g/kg of body weight per day up to 100 g/day) for at least two days and withdrawal of diuretics.

Minor Criteria Urine Volume < 500mg/dl Urine Sodium < 10 mmol/l Urine RBC < 50/hpf Serum Sodium concentration < 130 mmol/l

Page 45: Unusual cause of Renal failure

Type I hepatorenal syndrome is the more serious type; it is defined as at least a 50 percent lowering of the creatinine clearance to a value below 20 mL/min in less than a two week period or at least a twofold increase in serum creatinine to a level greater than 2.5 mg/dL

Type II hepatorenal syndrome is defined as less severe renal insufficiency than that observed with type I disease; it is principally characterized by ascites that is resistant to diuretics.

Page 46: Unusual cause of Renal failure

Liver and KidneyPotential Causes Tubular

InvolvementGlomerular involvement

Infections Sepsis, Leptospirosis, Brucellosis, B, EBV, Hepatitis B

Hepatitis B and CShistosoma Mansoni, HIV

Drugs and Toxins Nephrotoxic Drugs

Systemic Diseases Sarcoidosis, Sjogren Syndrome

SLE, vasculitis, cryoglobulinemian, Amyloidosis

Circulaory Failure Hypovolemia / shock

Congenital and Genetic

PCKD, Nephrolithiasis, Congenital hepatic fibrosis

Malignancy LukemiaLymphoma

Misc Fatty liver of pregnancy, Reyes Syndrome

Eclampsia, HELLP

Page 47: Unusual cause of Renal failure

References Comprehensive Clinical Nephrology , 3rd

edition- Feehaly Brenner and Recotor – The Kindey Gines, P, Schrier, RW. Renal failure in cirrhosis.

N Engl J Med 2009; 361:1279. Gines, P, Guevara, M, Arroyo, V, Rodes, J

Hepatorenal syndrome. Lancet 2003; 362:1819.

Page 48: Unusual cause of Renal failure

Hepatits B virus and Kidney Infection with hepatitis B virus (HBV) may be

directly associated with a variety of renal diseases, including polyarteritis nodosa, membranous glomerulonephritis, and membranoproliferative glomerulonephritis

Page 49: Unusual cause of Renal failure

Treatement Antiviral therapy targeted against HBV. Steroids and Immunosuppressants and/or

Plasma exchange in cases with RPGN features or vasculitis.

Conservative management in rest.

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Mukhtyar C, Guillevin L, Cid, MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Annals of Rheumatic Diseases April 2008.

Guillevin, L, Mahr, A, Cohen, P, et al. Short-term corticosteroids then lamivudine and plasma exchanges to treat hepatitis B virus-related polyarteritis nodosa. Arthritis Rheum 2004; 51:482.

Wen, YK, Chen, ML. Remission of hepatitis B virus-associated membranoproliferative glomerulonephritis in a cirrhotic patient after lamivudine therapy. Clin Nephrol 2006; 65:211.

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Thank you

Page 52: Unusual cause of Renal failure

Membranous nephropathy   HBV can induce the nephrotic syndrome due

to membranous nephropathy. It has been proposed, although not proven, deposition of HBeAg and anti-HBe is responsible

for the formation of pathogenic subepithelial immune deposits

Membranous nephropathy is most common in children and resolves spontaneously in many cases,

However, resolution is relatively uncommon in adults, most of whom appear to have progressive disease over time

Page 53: Unusual cause of Renal failure

Membranoproliferative glomerulonephritis 

The deposition of circulating antigen-antibody complexes in the mesangium and subendothelial space characterizes the membranoproliferative glomerulonephritis associated with HBV. Both HBsAg and HBeAg deposition have been implicated in

this

Some patients may have a membranoproliferative pattern due to mixed cryoglobulinemia; concurrent infection with

hepatitis C to be ruled out.

Page 54: Unusual cause of Renal failure

Polyarteritis nodosa — A large vessel vasculitis can be induced by HBV, in which circulating antigen-antibody complexes may be deposited in the vessels.

The vasculitis, which is called secondary PAN and has the same clinical features as idiopathic PAN that is not associated with HBV, typically occurs within four months after the onset of HBV infection

Page 55: Unusual cause of Renal failure

Diffuse Proliferative Glomerulonephritis A distinct form of glomerulonephritis common

to Autoimmune Disorder (eg SLE) Vasulitis Syndrome ( eg Wegeners

Granulomatosis) Infectious Process More than 50 % of glomeruli shows an increase in

mesangial, epithelial and enothelial proliferaive and inflammatory cells

If less than 50% focal proliferative

Page 56: Unusual cause of Renal failure

When to suspect DPGN Suspect DPGN in patients with SLE, infectious

disease processes, a recent streptococcal throat infection, or in patients with sinopulmonary disease who have recent onset of the following:

Hypertension Microscopic or gross hematuria Nonnephrotic or nephrotic range proteinuria or an

increase in proteinuria from baseline Serum creatinine of more than 0.4 mg/dL from the

reference range or the baseline Oligoanuria and symptoms of uremia in severe

cases of RPGN with crescent formation

Page 57: Unusual cause of Renal failure

A history of rash; photosensitivity; oral ulcers; arthralgias; arthritis; serositis; or a renal, neurologic, hematologic, or immunologic disorder suggests SLE as the primary disease.

A history of cough, dyspnea, hemoptysis, and renal disease suggests Goodpasture syndrome, but other pulmonary-renal syndromes must be ruled out, including SLE pneumonitis, Wegener granulomatosis, cryoglobulinemia, renal vein thrombosis with pulmonary embolism, legionella infection, and congestive heart failure.

Patients with Wegener granulomatosis present with sinopulmonary

Atypical Presentations of Ig A Nephropathy