glomerulonephritis (GN, nephritic syndrome)
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Introduction
Bilateral inflammation of renal glomeruli.
Etiology
(also seen expanded etiology of GN)
- idiopathic glomerulonephritis (GN)
- secondary glomerulonephritis (GN)
* most common etiologies
Pathology
- general
- infiltration of glomeruli with inflammatory cells
- proliferation of glomerular cells
- immunofluorescent staining
- granular pattern:
- linear pattern: Goodpasture's syndrome
- Pauci-immune immunofluorescence:
- idiopathic crescentic GN can have granular, linear or pauci-immune immunofluorescent pattern
Genetics
- upregulation of SCLY
Clinical manifestations
- general
- secondary signs/symptoms
- pharyngitis or dermatitis 2-3 weeks prior to onset of GN
- post-streptococcal GN
- arthritis
- lupus nephritis
- cryoglobulinemia
- polyarteritis nodosa
- hemoptysis
- Goodpasture's syndrome
- Wegener's granulomatosis
- sinusitis: Wegener's granulomatosis
- fever
- malar rash: lupus nephritis
- oral ulcers: lupus nephritis
- heart murmur: endocarditis
- palpable purpura:
- pharyngitis or dermatitis 2-3 weeks prior to onset of GN
- case with fatigue, arthralgia, myalgia, fever, hematuria, leucocyturia, proteinuria[3]
Laboratory
- urinalysis
- hematuria
- RBC casts, dysmorphic erythrocytes (acanthocytes*)
- specific but not sensitive for glomerulonephritis
- urine leukocytes variable[3]
- proteinuria variable[3]
- urine is 'cola' colored or smokey in appearance because of methemoglobin in acid urine
- serum chemistries
- complete blood count (CBC)
- 24 hour urine
- creatinine clearance generally diminished
- urine protein (generally not nephrotic range)
- blood cultures: rule out endocarditis
- serologies
- antistreptolysin O (ASO) titer, anti-DNAse antibodies
- anti-nuclear antibody (ANA), anti-dsDNA
- serum complement: serum C3, serum C4, CH50
- low levels with:
- normal levels with:
- hepatitis B serology, hepatitis C serology
- anti-neutrophil cytoplasmic antibody (ANCA)
- anti-glomerular basement membrane (anti-GBM)
- serum cryoglobulins
- HIV serology
* acanthocytes described in[3] as erythrocytes with 'Mickey Mouse' ears in the urine
Diagnostic procedures
- echocardiogram
- valvular lesions of endocarditis
- pericardial effusion
- kidney biopsy: immunofluorescent staining (when diagnosis is unclear)
- open lung biopsy or biopsy of nasal mucosa:
Radiology
- renal ultrasound is normal in glomerulonephritis
- chest X-ray
Differential diagnosis
* erythrocytes with 'Mickey Mouse' ears (acanthocytes) found in urine of patients with glomerulonephrites not to be confused with budding yeast on unstained urine microscopy
Management
- general considerations
- diuretics
- avoid ACE inhibitors & ARBs
- dialysis for severe renal failure
- diet
- sodium restriction < 2 g/day
- potassium restriction < 2 g/day
- follow-up
- long-term follow-up for chronic etiologies
- parameters of disease activity
- specific signs/symptoms of different etiologies
- specific therapies for different etiologies
- postinfectious glomerulonephritis: appropriate antibiotics
- immune-mediated glomerulonephritis
- IgA nephropathy
- MPGN, hepatitis C
- anti-GBM antibody disease: plasmapheresis
More general terms
More specific terms
- 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
Additional terms
References
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 541-42
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 604-608
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 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
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1295
- ↑ Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG. Pauci-immune necrotizing glomerulonephritis. Rheum Dis Clin North Am. 2010 Aug;36(3):559-72 PMID: https://pubmed.ncbi.nlm.nih.gov/20688250
- ↑ Hricik DE, Chung-Park M, Sedor JR Glomerulonephritis. N Engl J Med 1998; 339:888-899. Sept 24, 1998 <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/9744974 NEJM Full Text <Internet> http://www.nejm.org/doi/full/10.1056/NEJM199809243391306
- ↑ 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: https://pubmed.ncbi.nlm.nih.gov/26567243 Free PMC Article
- ↑ Sethi S, De Vriese AS, Fervenza FC. Acute glomerulonephritis. Lancet. 2022;399:1646-1663. PMID: https://pubmed.ncbi.nlm.nih.gov/35461559