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Description : Check out this medical presentation on amyloidosis, which is titled "Secondary Causes of the Nephrotic Syndrome", to know about glomerular diseases.

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Published on : May 06, 2021 | Views : 1223 | Downloads : 6

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Slide 1-

Secondary Causes of the Nephrotic Syndrome Sumit Kumar, MD Presbyterian Hospital Dallas Dallas, TX
Slide 2-

Normal Anatomy
Slide 3-

Classification of Glomerular Diseases Primary glomerular diseases Nephrotic Syndrome Non Immune Complex Immune Complex Nephritic syndrome Post infectious glomerulonephritis Ig A nephropathy / Henoch Schonlein purpura
Slide 4-

Classification of Glomerular Diseases Diseases Associated With Nephrotic Syndrome Monoclonal Immunoglobulin Deposition Disease (MIDD) Amyloidosis Light chain deposition disease Others Infections Deposition diseases Secondary FSGS Malignancies
Slide 5-

Classification of Glomerular Diseases Diseases Associated With Nephritic ± Nephrotic Syndrome or RPGN Immune Mediated: Lupus; cryoglobulin related; anti-GBM Pauci immune
Slide 6-

Proteinuria Proteinuria>150mg/24hr Dipstik Test: >1(+) False positive: gross hematuria Urine protein:urine creatinine: Normal <0.15 Ratio of 1 correlates with proteinuria of 1g/24hr Most accurate is 24 hr urine collection for protein and creatinine Pitfalls of spot protein testing: False positives and negatives
Slide 7-

Types of Proteinuria Glomerular Primary and Secondary Diseases Charge selectivity (as in MCD) Hemodynamic (severe HTN and CHF) Tubular Fanconi Syndrome, Tubulo-interstitial disease Overflow Monoclonal gammopathies Inflammatory Variants Transient: Usually disappears, No workup needed Orthostatic Associated with exercise, fever, stress
Slide 8-

Clinical Presentation of Glomerular Disease
Slide 9-

Clinical Presentation of Glomerular Disease
Slide 10-

Clinical Pathways for the Pathogenesis of Glomerular disease Glomerular Disease Asymptomatic hematuria Macroscopic Hematuria Nephritic Syndrome Nephrotic Syndrome RPGN Chronic Glomerulonephritis ESRD
Slide 11-

Common Causes of Nephrotic Syndrome
Slide 12-

Secondary Causes of the Nephrotic Syndrome Hereditary: Rare Infectious: Viral: HBV, HCV, HIV Bacterial: 2 syphilis, SBE Protozoan: Ch malaria Immunologic: SLE Drugs: Gold, Penicillamine, NSAIDs Metabolic: Diabetes Mellitus Amyloidosis Neoplasms: Myeloma Solid e.g. colon, lung, breast Lymphoma, leukemia Miscellaneous: Massive obesity, sleep apnea syndrome, chronic reflux nephropathy (FSGS)
Slide 13-

Secondary Glomerular Diseases Diabetic Nephropathy Lupus Nephritis Secondary focal segmental glomerulosclerosis Secondary membranous glomerulopathy Membranoproliferative Glomerulonephritis Paraproteinemia Collagen Vascular Disease Malignancy associated
Slide 14-

Doc…..I think I am not well!! Periorbital edema Pedal edema Weight gain
Slide 15-

Approach to the Patient with Nephrotic Syndrome Make the diagnosis Proteinuria > 3.5 g/1.73 m2 Hypoalbuminemia Edema Lipuria Hypercholesterolemia Screen the patient for secondary etiologies
Slide 16-

Assessment of a Nephrotic patient History: medications, drugs, surgeries, infections, obesity, travel, family history Serologies Hepatitis B and C, HIV ANA, C3, C4, SPEP, UPEP, Cryoglobulins VDRL, ASO titer Imaging Chest Xray Renal sonogram
Slide 17-

Diabetic Nephropathy
Slide 18-

Prevalent counts & adjusted rates, by primary diagnosis Point prevalent ESRD patients; Medical Evidence form data; rates adjusted for age, gender, & race.
Slide 19-

Projected growth of the U.S. diabetic population, by race White Black Other 1978 4,325,230 824,354 117,175 1990 6,475,204 1,319,594 385,756 2000 8,786,661 2,005,805 773,134 2030 18,441,647 5,539,283 2,941,056 Prevalence rates of diabetes in 1980–1998 obtained from National Health Interview Survey (NHIS) data. These data are linearly extrapolated to obtain prevalent rates for 1978–2030, & estimates are then multiplied by census projections to obtain estimated numbers of individuals with diabetes for 1978–2030.
Slide 20-

Onset of Diabetes Functional Changes  GFR Reversible albuminuria  Kidney size 0 2 5 Structural Changes GBM thickening Mesangial expansion 10 -30 Onset of Proteinuria Onset of Hypertension 13-25 GFR 15-40 ESRD Incipient Nephropathy Hyperfiltration  Blood Pressure Poor glycemic control Time (yrs) The Natural History of Diabetic Nephropathy in IDDM
Slide 21-

Natural History of Diabetic Nephropathy in NIDDM- Lessons learnt from the Pima Indians Nelson et al NEJM 1996;335:1636-1642
Slide 22-

Natural History of Diabetic Nephropathy in NIDDM- Lessons learnt from the Pima Indians Nelson et al NEJM 1996;335:1636-1642
Slide 23-

Diabetic Nephropathy
Slide 24-

Pathology of Diabetic Nephropathy
Slide 25-

Kimmelstein Wilson Nodular Sclerosis
Slide 26-

Major Therapeutic Maneuvers to Slow Loss of GFR in Diabetic Nephropathy Hyperglycemia Glomerulosclerosis Normotension Euglycemia Protein restriction Lipid Management Weight loss, exercise, smoking cessation ACEi, ARB
Slide 27-

Lupus Nephritis
Slide 28-

Lupus Nephritis Renal involvement Early Course: 30-50% of unselected patients Later Course: 60-80% Most patients present with proteinuria Hypertension ± Hyperkalemic renal tubular acidosis
Slide 29-

Clinical Features of Lupus Nephritis
Slide 30-

Lab tests in Lupus Nephritis ANA, Anti DNA antibody, Anti Smith antibody Anemia of moderate degree, Coombs + in minority, severe hemolytic anemia – rarely Leukopenia, thrombocytosis Hypocomplementemia: C4 and C1q are depressed more than C3 suggesting classic pathway activation (never occurs in idiopathic MPGN Antiphospholipid antibody - ⅓ - ½ of patients with lupus nephritis Renal arterial, venous and glomerular cap thrombosis, Libman-Sacks arthritis, cerebral thrombosis
Slide 31-

Diagnosis and Differential Diagnosis Suspect in Middle aged, nephrotic male Idiopathic membranous nephropathy in a young woman Routine screening of all nephrotic patients with ANA Differential: Rheumatoid arthritis; Henoch Schonlein purpura; Ig A nephropathy; vasculitis
Slide 32-

Clinicopathologic Correlations in Lupus Nephritis
Slide 33-

WHO Class II Lupus Nephritis Mesangial disease – 10-25% of all biopsies Mesangial expansion Clinically mild disease – non nephrotic proteinuria; normal renal function
Slide 34-

WHO Class III Lupus Nephritis Focal Proliferative 20-35% of biopsies Focal necrosis Clinically mild disease – proteinuria; hematuria ±
Slide 35-

WHO Class IV Lupus Nephritis Diffuse proliferative (DPGN) 35-60% of biopsies Hypercellularity Intense inflammation Clinically – hematuria, red cell casts, proteinuria, hypertension, acute renal failure. Most amenable to treatment Rx: NIH Protocol
Slide 36-

WHO Class V Lupus Nephritis Membranous 10-15% of biopsies Silver positive “spikes” – subepithelial Clinically nephrotic, often normal renal function Rx: Difficult to treat; Steroids; Ponticelli protocol
Slide 37-

Secondary Focal Sclerosing Glomerulosclerosis (FSGS)
Slide 38-

Focal Segmental Glomerulosclerosis
Slide 39-

Focal Segmental Glomerulosclerosis
Slide 40-

Classification of Secondary FSGS Severe Nephrotic Syndrome Drugs: heroin; NSAIDs Viruses: Hep B, HIV, parvo Non Nephrotic proteinuria Reduced Mass Solitary kidney, allograft, surgical ablation, renal dysplasia, agenesis, segmental hypoplasia Non Nephrotic Proteinuria Scarring VUR, hypertensive nephrosclerosis, post infectious or inflammatory; lupus nephritis; vasculitides Hyperfiltration Obesity, sickle cell nephropathy, congenital cyanotic heart disease Other Causes Malignancies: lymphomas Misc: sarcoidosis, radiation nephritis; Charcot-Marie-Tooth
Slide 41-

Secondary FSGS HIV associated Nephropathy (HIVAN) Vesicoureteric reflux Obesity
Slide 42-

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Slide 43-

HIV associated Nephropathy Massive proteinuria Micro-hematuria Azotemia Rapid progression to ESRD African American Patients CD4 count low Normotensive Sonogram
Slide 44-

HIV associated Nephropathy Natural History Malignant course ESRD within 3 to 4 months Less common now than 10 years ago Differential Diagnosis Heroin associated Nephropathy Options Dialysis HIV treatment Transplantation??
Slide 45-

Secondary FSGS Vesicoureteric reflux May not occur for several years Often a poor prognostic sign – strong correlation between extent of glomerular involvement and the magnitude of proteinuria and GFR decline\ Hypertension occurs late Histology: Hyalinosis in unscarred areas of the kidney or in the contralateral normal kidney
Slide 46-

Secondary FSGS Obesity Proteinuria is fairly common (upto 40%) Likely to be part of the endothelial dysfunction syndrome a.k.a. dysmetabolic syndrome X Remission of proteinuria often occurs with weight reduction Association with sleep apnea
Slide 47-

Paraproteinemia
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Slide 49-

Case Presentation - Amyloidosis B.J.A., a 56 AA female with a long standing h/o HTN for the past 30 years was in good health until about 8 weeks prior to presentation. Insidious onset of fatigue; 5 kg lost over a 2 m period. She had a baseline Cr of 1.6 three m prior. About a week prior, she was started on Atenolol and HCTZ for recent difficulty in controlling her BP. at that time, a Captopril renography was performed after Captopril 25 mg was given - Study was negative. Over the course of the next week, her symptoms of fatigue worsened and she was admitted to DGH with progressively worsening SOB, 3-4 pillow orthopnea, and 2 episodes of syncope. Hypertension for 30 yrs Nifedipine XL 90 mg po qd, HCTZ 25 po qd, Atenolol 25 mg po qd and ASA HOPI PMH Meds
Slide 50-

Case Presentation - Amyloidosis 94/56; Wt 54 kg nd Ht 5’2” Eyes revealed Gr II HTNsive retinopathy JVP : 13 cm Chest had rales on the bases Precordial heave and a PSM was heard at the apex 3/6 rad to axilla Liver was 3cm BCM, Firm and non tender Urine: SG 1.020, Protein 2+, Normal sediment Chem-7: Na 135, K 4.3, Cl 96, HCO3, BUN 76 and Cr 6.7 CXRMild Pulmonary edema EKG: NSR, LVE, LAFB PE Labs
Slide 51-

Case Presentation - Amyloidosis SG Catheter was placed - CI:3.8, PCWP:22, SVR:1535, PAP:48/20 She was started on dobutamine and dopamine with no appreciable change in hemodynamics. On day 3, HD was started for worsening azotemia, nausea & vomiting. Other w/up included Cardiac Echo: Restrictive cardiomyopathy with EF 25% Renal US: Normal sized kidneys with increased echogenicity IE: Monoclonal l spike in the g region on serum and urine immunoelectropheresis Oral fat pad biopsy Rectal Biopsy: revealed a single vessel with amyloid deposits staining + with Congo Red with apple-green birefringence under polarized light She died 40 days later due to intra and post dialytic hypotension complicated by malignant ventricular arrhythmias Hospital Course
Slide 52-

Amyloidosis – an Infiltrative Disease
Slide 53-

Symptoms & Signs in Amyloidosis Median Wt loss ~ 23 # Renal insufficiency in 45% usually without hypertension Proteinuria in 73%
Slide 54-

Differentiate between Primary and Secondary Amyloidosis H/O inflammatory or infectious disease Family h/o amyloidosis, organ distribution Presence of a paraprotein in primary amyloid ALA pts may have one or more of the following Autonomic and peripheral neuropathy & CTS Restrictive cardiomyopathy Non thrombocytopenic purpura Large joint arthropathy Cutaneous plaques and nodules and macroglossia Isolated factor IX and X deficiency
Slide 55-

Amyloidosis Clinical suspicion Immmunoelectropheresis Tissue Biopsy Bone Marrow Biopsy 123I-SAP Scintigraphy Tissue Biopsy Abdominal Fat Pad 60-90% Rectal Biopsy 50-80% Bone Marrow 30-50% in primary ALA Bone Marrow 80% in FMF Gingiva 60% Skin 50%
Slide 56-

Outcome in AL Amyloidosis Median Survival time of 229 pts was 12 m and less than 25% were alive at 3 yrs. Cardiomyopathy and arrhythmias accounted for ~ 40-50% of the deaths. Multivariate analysis of 168 patients - Cardiomyopathy, Urine LC, Hepatomegaly & MM affected survival in the 1st year.
Slide 57-

Entities covered ….. General discussion Diabetic Nephropathy Lupus Nephritis Secondary causes for FSGS: HIVAN Amyloidosis
Slide 58-

Entities we did not cover….. Hepatitis C related Glomerulonephritis Secondary causes of membranous nephropathy Rheumatologic causes of nephrotic syndrome Malignany related nephrotic syndrome
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