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glomerulonephritis (GN, nephritic syndrome)

Bilateral inflammation of renal glomeruli. Etiology: (also seen expanded etiology of GN) 1) idiopathic glomerulonephritis (GN) - membranoproliferative glomerulonephritis (MPGN) - idiopathic crescentic glomerulonephritis - IgA nephropathy* - fibrillary glomerulonephritis - immunotactoid glomerulonephritis 2) secondary glomerulonephritis (GN) a) infection - endocarditis* - post-streptococcal glomerulonephritis* - visceral abscess - sepsis* b) connective tissue disease - systemic lupus erythematosus (lupus nephritis)* c) vasculitis (pauci-immune glomerulonephritis)* - Wegener's granulomatosis - polyarteritis nodosa - microscopic polyangiitis d) anti-glomerular basement membrane disease - Goodpasture's syndrome - thin glomerular basement membrane disease e) Henoch Schonlein purpura* f) cryoglobulinemia g) multiple myeloma h) Alport's syndrome i) thrombotic microangiopathy - hemolytic uremic syndrome - thrombotic thrombocytopenic purpura j) pharmaceutical agents * most common etiologies Pathology: 1) general a) infiltration of glomeruli with inflammatory cells b) proliferation of glomerular cells 2) immunofluorescent staining a) granular pattern: 1] post-streptococcal GN 2] lupus nephritis 3] cryoglobulinemia [3] 4] IgA nephropathy [3] 5] membranoproliferative glomerulonephritis [3] 6] endocarditis b) linear pattern: Goodpasture's syndrome c) Pauci-immune immunofluorescence: - ANCA-associated glomerulonephritis - Wegener's granulomatosis - polyarteritis nodosa - others d) idiopathic crescentic GN can have granular, linear or pauci-immune immunofluorescent pattern Genetics: (biochemistry) - upregulation of SCLY Clinical manifestations: 1) general - dyspnea: pulmonary edema - edema: lower extremities, periorbital - oliguria & dark urine - hypertension - renal failure [3] 2) secondary signs/symptoms a) pharyngitis or dermatitis 2-3 weeks prior to onset of GN - post-streptococcal GN b) arthritis a) lupus nephritis b) cryoglobulinemia c) polyarteritis nodosa c) hemoptysis a) Goodpasture's syndrome b) Wegener's granulomatosis d) sinusitis: Wegener's granulomatosis e) fever - endocarditis - lupus nephritis f) malar rash: lupus nephritis g) oral ulcers: lupus nephritis h) heart murmur: endocarditis i) palpable purpura: - Henoch Schonlein purpura - cryoglobulinemia Laboratory: 1) urinalysis a) hematuria b) RBC casts, dysmorphic erythrocytes - specific but not sensitive for glomerulonephritis c) urine leukocytes variable [3] d) proteinuria variable [3] e) urine is 'cola' colored or smokey in appearance because of methemoglobin in acid urine 2) serum chemistries a) serum urea nitrogen b) serum creatinine 3) complete blood count (CBC) 4) 24 hour urine a) creatinine clearance generally diminished b) urine protein (generally not nephrotic range) 5) blood cultures: rule out endocarditis 6) serologies a) antistreptolysin O (ASO) titer, anti-DNAse antibodies b) anti-nuclear antibody (ANA), anti-dsDNA c) serum complement: serum C3, serum C4, CH50 1] low levels with: a] post-streptococcal GN b] endocarditis c] lupus nephritis d] membranoproliferative GN e] cryoglobulinemia (hepatitis C) 2] normal levels with: a] vasculitis b] IgA nephropathy - Henoch Schonlein purpura c] idiopathic crescentic GN d] anti-glomerular basement membrane disease - Goodpasture's syndrome d) hepatitis B serology, hepatitis C serology 1] cryoglobulinemia 2] polyarteritis nodosa 3] membranoproliferative GN e) anti-neutrophil cytoplasmic antibody (ANCA) 1] Wegener's granulomatosis 2] polyarteritis nodosa, 3] idiopathic crescentic GN f) anti-glomerular basement membrane (anti-GBM) -> Goodpasture's syndrome g) serum cryoglobulins h) HIV serology Special laboratory: 1) echocardiogram a) valvular lesions of endocarditis b) pericardial effusion 2) kidney biopsy: immunofluorescent staining (when diagnosis is unclear) 3) open lung biopsy or biopsy of nasal mucosa: -> Wegener's granulomatosis Radiology: 1) renal ultrasound is normal in glomerulonephritis 2) chest X-ray a) pulmonary edema b) pleural effusion c) pulmonary hemorrhage in Goodpasture's syndrome d) pulmonary infiltrates, pulmonary nodules or cavitary lesions in Wegener's granulomatosis Differential diagnosis: - nephrotic syndrome Management: 1) general considerations a) diuretics - volume overload - hypertension b) avoid ACE inhibitors & ARBs c) dialysis for severe renal failure d) diet - sodium restriction < 2 g/day - potassium restriction < 2 g/day e) follow-up - long-term follow-up for chronic etiologies - parameters of disease activity - urinalysis: proteinuria, RBC - serum chemistries: - BUN, serum creatinine, serum electrolytes - serum complement; serum C3, serum C4, CH50 - specific signs/symptoms of different etiologies 2) specific therapies for different etiologies a) postinfectious glomerulonephritis: appropriate antibiotics b) immune-mediated glomerulonephritis - SLE, vasculitis, anti-GBM disease cryoglobulinemia - corticosteroids, fish oil, mycophenolate c) IgA nephropathy - corticosteroids, cyclophosphamide, mycophenolate d) MPGN, hepatitis C - corticosteroids, dipyridamole, aminosalicylate e) anti-GBM antibody disease: plasmapheresis

Interactions

disease interactions

Related

etiology of glomerulonephritis glomerulus

Specific

anti-glomerular basement membrane [GBM] disease chronic glomerulonephritis crescentic glomerulonephritis (rapidly progressive glomerulonephritis) drug-induced glomerulonephritis (GN) fibrillary glomerulonephritis immunotactoid glomerulonephritis lupus nephritis membranoproliferative glomerulonephritis membranous glomerulonephritis; mesangiocapillary glomerulonephritis (membranous nephropathy) mesangioproliferative glomerulonephritis pauci-immune glomerulonephritis postinfectious glomerulonephritis; infection-related glomerulonephritis sclerosing glomerulonephritis

General

glomerulonephropathy; glomerulopathy nephritis

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 541-42
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 604-608
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1295
  5. Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG. Pauci-immune necrotizing glomerulonephritis. Rheum Dis Clin North Am. 2010 Aug;36(3):559-72 PMID: 20688250
  6. Hricik DE, Chung-Park M, Sedor JR Glomerulonephritis. N Engl J Med 1998; 339:888-899. Sept 24, 1998 PMID: 9744974 NEJM Full Text http://www.nejm.org/doi/full/10.1056/NEJM199809243391306
  7. Sethi S, Haas M, Markowitz GS et al Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. J Am Soc Nephrol. 2016 May;27(5):1278-87. PMID: 26567243 Free PMC Article