glomerulonephritis (GN, nephritic syndrome)

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^[1]^[2]^[3]^[4]^[5]^[6]^[7]

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:
  - ANCA-associated glomerulonephritis
    - Wegener's granulomatosis
    - polyarteritis nodosa
    - others
- idiopathic crescentic GN can have granular, linear or pauci-immune immunofluorescent pattern

Genetics

upregulation of SCLY

Clinical manifestations

general
- dyspnea: pulmonary edema
- edema: lower extremities, periorbital
- oliguria & dark urine
- hypertension
- renal failure^[3]
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
  - endocarditis
  - lupus nephritis
- malar rash: lupus nephritis
- oral ulcers: lupus nephritis
- heart murmur: endocarditis
- palpable purpura:
  - Henoch Schonlein purpura
  - cryoglobulinemia

Laboratory

urinalysis
- hematuria
- RBC casts, dysmorphic erythrocytes
  - 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
- serum urea nitrogen
- serum creatinine
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:
    - post-streptococcal GN
    - endocarditis
    - lupus nephritis
    - membranoproliferative GN
    - cryoglobulinemia (hepatitis C)
  - normal levels with:
- hepatitis B serology, hepatitis C serology
- anti-neutrophil cytoplasmic antibody (ANCA)
- anti-glomerular basement membrane (anti-GBM)
  - Goodpasture's syndrome
- serum cryoglobulins
- HIV serology

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:
- Wegener's granulomatosis

Radiology

renal ultrasound is normal in glomerulonephritis
chest X-ray

Differential diagnosis

nephrotic syndrome

Management

general considerations
- diuretics
  - volume overload
  - hypertension
- 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
    - urinalysis: proteinuria, RBC
    - serum chemistries:
      - BUN, serum creatinine, serum electrolytes
    - serum complement; serum C3, serum C4, CH50
  - specific signs/symptoms of different etiologies
specific therapies for different etiologies
- postinfectious glomerulonephritis: appropriate antibiotics
- immune-mediated glomerulonephritis
  - SLE, vasculitis, anti-GBM disease cryoglobulinemia
  - corticosteroids, fish oil, mycophenolate
- IgA nephropathy
  - corticosteroids, cyclophosphamide, mycophenolate
- MPGN, hepatitis C
  - corticosteroids, dipyridamole, aminosalicylate
- anti-GBM antibody disease: plasmapheresis

More general terms

More specific terms

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 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
↑ 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://www.ncbi.nlm.nih.gov/pubmed/20688250
↑ Hricik DE, Chung-Park M, Sedor JR Glomerulonephritis. N Engl J Med 1998; 339:888-899. Sept 24, 1998 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/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://www.ncbi.nlm.nih.gov/pubmed/26567243 Free PMC Article

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