Glomerulonephritis. Nephrology .pptx

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1. Glomerulopathies Dr aldosoky abd elaziz

2. Glomerulopathies The presentation of glomerular diseases can be nephrotic syndrome, characterized by proteinuria >3.5 g/d, and accompanied by hypoalbuminemia , hyperlipidemia , and edema. On the other hand is the nephritic syndrome, characterized by hematuria , hypertension, edema, and renal insufficiency.

3. General TTT Proteinuria treatment with ACE inhibitors or ARBs to reduce the intraglomerular pressure. Electrolytes and creatinine should be monitored to document stability of renal function and potassium. Edema and volume overload can usually be effectively managed with diuretics combined with salt restriction.

4. The hyperlipidemia associated with the nephrotic syndrome responds to dietary modification and HMG- CoA reductase inhibitors. The nephrotic syndrome produces a hypercoagulable state and can predispose to thromboembolic complications. Deep venous thrombi and renal vein thrombosis may occur and should be treated with heparin anticoagulation followed by long-term warfarin therapy.

5. Acute form of renal vein thrombosis usually occurs in younger patients, with flank pain and macroscopic hematuria , which can be severe in the acute onset of thrombosis.

6. Minimal Change Disease The most common cause of the nephrotic syndrome in children. Sudden onset of proteinuria with hypertension and edema as well as the full nephrotic syndrome, although renal insufficiency is unusual. MCD may accompany certain malignancies (Hodgkin disease and solid tumors being the most common).

7. The kidney biopsy reveals normal glomeruli on light microscopy and negative immunofluorescence . Electron microscopy shows effacement of the foot processes as the only histological abnormality. Corticosteroids are the treatment of choice, leading to complete remission of proteinuria in most cases. MCD secondary to Hodgkin lymphoma is frequently resistant to steroids and will remit with cure of the primary disease.

8. Focal Segmental Glomerulosclerosis Presentation is with the nephrotic syndrome, hypertension, and renal insufficiency. Obesity, HIV and intravenous drug abuse. The kidney biopsy reveals focal and segmental sclerosis of glomeruli under light microscopy. Immunofluorescence shows staining for C3 and IgM in areas of sclerosis. Electron microscopy shows effacement of the podocyte foot processes. TTT with steroids if not effective immune suppressive therapy.

9. Membranous Nephropathy Membranous nephropathy (MN) usually presents with the nephrotic syndrome or heavy proteinuria . MN are associated with SLE, viral hepatitis, syphilis, or solid organ malignancies. Medications such as gold and penicillamine can also induce this process.

10. Kidney biopsy shows thickening of the glomerular basement membrane on light microscopy, with “spikes” on silver stain , representing areas of normal basement membrane interposed between subepithelial deposits. IgG and C3 on immunofluorescence .

11. TTT : ttt underkying cause. TTT with steroids if no response immune suppressive in the following cases : reduced GFR, male gender, age > 50, hypertension, severe nephrotic syndrome ( proteinuria > 10 g/d).

12. Membranoproliferative Glomerulonephropathy Primary idiopathic membranoproliferative glomerulonephropathy (MPGN) is uncommon. Hepatitis C accounts for most cases of secondary MPGN, frequently in association with cryoglobulinemia . Cryoglobulins are single or mixed immunoglobulins that undergo reversible precipitation at low temperatures.

13. This may result in a clinical syndrome of systemic inflammation (most commonly affecting the kidneys and skin) caused by cryoglobulin -containing immune complexes. acrocyanosis , retinal hemorrhage, severeRaynaud phenomenon with digital ulceration, livedo reticularis , purpura , arthtritis …….and off course our topic kindey involvement in the form of MPGN. Avoid cold, search for HCV and ttt it…..in severe acute attacks plasmapheresis .

14. MPGN عودة لل MPGN can present with the nephrotic syndrome, nephritic syndrome. Complement levels (C3, C4) are frequently low and a C3-nephritic factor . C3 nephritic factor is an autoantibody to the C3 convertase which increases the half-life of the convertase ………> DECREASED COMPLEMENT LEVELS.

15. The kidney biopsy shows mesangial proliferation and hypercellularity on light microscopy. “tram-track” appearance on silver stain. Immunofluorescence and electron microscopy can show subendothelial (type I) or intramembranous (type II) deposits. TTT : ttt of the underlying cause, steroids, immune suppressive.

16. IgA Nephropathy The disease is the result of the deposition of circulating immune complexes “ IgA ” in the kidney. Two common presentations of patients with IgA nephropathy are episodic gross hematuria and persistent microscopic hematuria . Recurrent episodic hematuria is the most common clinical presentation in patients…….>so most likely to appear on the test that way.

17. Mostly present with a nephritic picture. Eighty percent of these episodes are associated with upper respiratory tract infections, mainly acute pharyngotonsillitis . How to differentiate between it and poststreptococcal glomerulonephritis ??

18. Timing..timing…timing…timing! Gross hematuria usually appears simultaneously or within the first 48-72 hours after pharyngitis . IgA and C3 deposits on immunofluorescence . TTT : steroids…..immune suppressive.

19. Anti-GBM antibody disease Goodpasture's syndrome. Circulating antibody to the alpha-3 chain of type IV collagen is deposited in the basement membrane of alveoli and glomeruli . ONLY KIDNEY AND LUNG….LUNG MEANS LUNG NOT RESPIRATORY SO IF YOU HAVE SOMEONE WITH RENAL INVOLVEMENT AND SINUSITIS THIS IS NOT GOOD PASTTURE…..SO WHAT DISEASE??

20. Rapidly progressive renal failure often with concurrent pulmonary involvement in the form of alveolar hemorrhage. A nephritic picture predominates, with dysmorphic RBCs and RBC casts in the urine. Presence of anti-GBM antibody in the serum. The goal of therapy is to clear the pathogenic antibody while suppressing new production.

21. Daily total volume plasmapheresis for approximately 14 days in conjunction with cyclophosphamide and glucocorticoids .

22. Postinfectious Glomerulonephropathy classically presents with the nephritic syndrome of hematuria , hypertension, edema, and renal insufficiency. Proteinuria may be present and is usually in subnephrotic range. Patients with nephrotic -range proteinuria in the acute phase or persistent heavy proteinuria have a worse prognosis.

23. Occurs after a latent period of 2 to 4 weeks from onset of pharyngitis or skin infection. IgA neprhopathy ?????????? Low complement levels are usually seen. Elevated Antistreptolysin -O (ASO) titers may and anti- DNaseB antibodies and antihyaluronidase . Kidney biopsy reveals subepithelial humps on light and electron microscopy corresponding to the deposits on immunofluorescence ( IgG , C3).

24.  Complement levels return to normal in 6-8 weeks. Hematuria usually resolves within 3-6 months . Proteinuria usually disappears in 6 months. Specific therapy for streptococcal infection is an important part of the therapeutic regimen. Treat patients, family members, and any close personal contacts who are infected. Throat cultures should be performed on all these individuals. 

25. Treat with oral penicillin G or with erythromycin for patients allergic to penicillin. This helps prevent nephritis in carriers and helps prevent the spread of nephritogenic strains to others. Don’t forget to treat the complications of nephritic syndrome as in any other condition e.g HTN , elevated Bun/ Creat .