View
2.019
Download
4
Embed Size (px)
Citation preview
Physicians Meet – M2Case Presentation
Prof. Dr. S. Sundar’s Unit
Presented by Dr. Deepu Sebin
Ponnammal , 55 yr old female House wife First admitted with complaints of
Abdominal distention – 1 month Swelling of legs – 1 month
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
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
General Examination PR – 80/min BP - 110/70 mmHg JVP – elevated No pallor/cyaosis/clubbing/lymphadenopathy BPPE + No Signs of liver cell failure
Systems P/A
Distented Free Fluid + No organomegaly BS +
Chest Clear AEBE
CVS S1 S2 normal
CNS NFND PEARL
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
Urine Rountine Albumin - + Sugar – Nil Depostis – 1 -4 pus cells
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
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
HbSAg – positive Anti HCV – negative HIV – negative OGDscopy – Grade 2 fundal varices
Impression : DCLD with Portal Hypertension HBV Related
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
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
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
Urine Rountine Albumin - + Sugar – Nil Depostis –1-3 pus cells 10-12 RBCs
DCLD – Portal Hypertension , HBV related AKI - ? Cause to r/o HRS Non oliguric
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 )
Plasma Urea / Creatine Ratio – 37.2 (>30 prerenal)
Urine to plasma creatine ratio - 24 (<20 ATN , >40 pre renal )
Urine Creatininre – 43mg/dl
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
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
In view of the Elevated renal parameters Which is static Not resolving with conservative management Proteinuria
Proceeded with Renal Biopsy after Nephrology Review
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.
Full House pattern IgM, IgG, IgA, C3c, and C1q
Possibility of Lupus Nephritis, vasculitis and other connective tissue disorders with DPGN – full house pattern.
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)
HBsAg – Postive HBeAg – Positive HBV DNA levels – > 20,000 IU/mL
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
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
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.
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
Focal Nephritic
Diffuse Nephritic
Nephrotic
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.
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
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.
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
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)
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 !
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.
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)
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
Renal Failure in Cirrhosis Hepatorenal Syndrome Psuedohepaorenal Syndrome
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
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.
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
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.
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
Treatement Antiviral therapy targeted against HBV. Steroids and Immunosuppressants and/or
Plasma exchange in cases with RPGN features or vasculitis.
Conservative management in rest.
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.
Thank you
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
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.
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
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
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
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