Basics
Description
- Syndrome characterized by:
- Hematuria
- Proteinuria
- Red blood cell casts
- Hypertension
- Renal insufficiency
- Common pathway of multiple diseases resulting in intraglomerular inflammation and cellular proliferation
- Contributing factors:
- Genetics
- Infectious
- Rheumatologic
- Leading to antibody deposition:
- Antibody attaches to glomerular antigen (native or implanted).
- Circulating antigen-antibody complex deposited
- Causing an influx and activation of inflammatory mediators:
- Leukocytes, complement, cytokines
- Cell-mediated immune mechanisms
- Results in glomerular dysfunction
- Persistent inflammation that can lead to scarring and permanent damage.
Etiology
- Postinfectious:
- Poststreptococcal glomerulonephritis (PSGN):
- Occurs 7-21 days after Streptococcal pharyngitis or skin infection
- Highest prevalence in ages 2-14 and the elderly
- Male predominance
- Ranges from asymptomatic hematuria to oliguric renal failure
- Can follow other bacterial, fungal, viral, or parasitic infections
- IgA nephropathy (Berger disease):
- Most common in men in the 3rd and 4th decades of life
- Possibly related to increased production of IgA after infection usually a URI
- Henoch-Sch ¶nlein purpura (HSP) has IgA nephropathy but affects a younger age and has systemic symptoms
- Rapidly progressive glomerulonephritis (RPGN):
- Can destroy renal function in days
- Crescentic deposits in glomeruli destroy function.
- Pauci-immune (small vessel vasculitides):
- Often antineutrophil cytoplasmic antibody (ANCA)-positive
- Can involve other areas (i.e., lungs, skin)
- Wegener granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
- Immune complex deposits:
- Postinfectious
- Endocarditis associated
- Systemic disease (i.e., systemic lupus erythematosus [SLE], HSP)
- Anti-glomerular basement membrane (GBM) deposits:
- Older patients of age >60
- Goodpasture disease with pulmonary involvement
- Membranoproliferative glomerulonephritis (MPGN):
- Complement deposits in basement membrane
- Hepatitis C
- Non-Hodgkin lymphoma
- Occult infection
Diagnosis
Signs and Symptoms
- Cardinal signs:
- Edema:
- Owing to renal salt and water retention
- Periorbital
- Ascites
- Pleural effusion
- HTN
- Oliguria
- Azotemia
- CHF
- Renal failure
- Nonspecific manifestations:
- Fatigue
- Weight loss
- Abdominal pain
- Nausea/vomiting
- Autoimmune disorders:
- Arthralgias
- Arthritis
- Rash
- Fever
- Goodpasture syndrome:
- Wegner granulomatosis:
- Purulent rhinorrhea/sinus pain
- Arthritis/arthralgias
- Hemoptysis
- HSP:
- Abdominal pain
- Purpura
- Arthritis
- Pauci-immune RPGN is common in this population (hematuria, proteinuria, and elevated CR).
- Urgent diagnosis and biopsy are indicated as this may progress to ESRD.
- Consult nephrology to discuss steroids, cyclophosphamide, and plasma exchange.
Essential Workup
Urinalysis for:
- Hematuria, proteinuria, and RBC casts
Diagnosis Tests & Interpretation
Lab
- Electrolytes, BUN, Creatinine, GFR:
- Renal function
- Hyperkalemia
- Albumin, total protein:
- Varying degrees of hypoalbuminemia depending on clinical process
- CBC:
- Anemia secondary to chronic renal disease, neoplasm, Goodpasture, and Wegener
- With or without elevated WBC in infections
- PT, PTT:
- Coagulation factors consumed in certain types of GN
- Labs to be considered for consultants:
- Cultures-throat, skin, blood
- 24-hr urine collection-protein, urine electrolytes
- Streptozyme or antistreptolysin O titer
- Complement levels (C1, C3, C4, CH50)-reduced in PSGN, MPGN, SLE
- ANA, rheumatoid factor-connective tissue diseases
- ESR and CRP inflammatory markers
- Anti-GBM-Goodpasture
- c-ANCA-Wegener; p-ANCA-pauci-immune
- Anti-DNA antibodies (SLE)
- Hepatitis B and C serologies
- HIV
Imaging
- Renal ultrasound (if GFR is decreased):
- Kidney size predictor of potential reversibility of disease, alternative diagnosis (i.e., neoplasm, stone)
- Chest radiograph (CXR): Heart size, pulmonary edema, or hemorrhage
Diagnostic Procedures/Surgery
- Renal biopsy: Discern primary glomerulopathies vs. other causes
- Cystoscopy: If concern for bladder neoplasm
Differential Diagnosis
- Hematologic:
- Sickle cell disease
- Coagulopathy
- Renal:
- Infectious
- Malformation
- Neoplasm
- Ischemic
- Trauma
- Vasculitis
- Postrenal:
- Mechanical (i.e., stones, reflux, obstruction, catheterization)
- Inflammatory (i.e., cystitis, prostatitis, epididymitis, endometriosis, periurethritis)
- Neoplasm
- Factitious:
- Food
- Drugs
- Pigmenturia (i.e., myoglobin, porphyria, hemoglobin)
- Vaginal bleeding
Treatment
Pre-Hospital
- Supportive
- ABCs and fluid restriction in stable patients with significant edema.
Initial Stabilization/Therapy
ABCs: Airway, breathing, circulation
Ed Treatment/Procedures
- Treatment mainly supportive care:
- BP control: <125/75 mm Hg
- Loop diuretics
- ACE inhibitor for maintenance
- Treat hypertensive emergencies
- Dialysis:
- Fluid overload
- Hyperkalemia
- Uremia
- PSGN:
- Supportive care
- Usually resolves spontaneously
- No benefit to antibiotics
- IgA nephropathy:
- Supportive care
- Immunosuppressives if inflammation on biopsy
- Variable course, most recover but may relapse
- RPGN:
- Can irreversibly destroy renal function in days
- Emergently consult nephrologist to discuss starting potentially toxic therapies.
- Immunosuppressives and high-dose steroids:
- Methylprednisolone and prednisone
- Cyclophosphamide
- Rituximab
- Plasmapheresis for anti-GBM antibody
- MPGN:
- Treat underlying disease if known.
- Emergently consult nephrologist to discuss starting potentially toxic therapies.
- May include plasma exchange, cyclophosphamide, and/or steroids
Medication
- Benazepril: 5-40 mg PO daily (or any other ACE inhibitor)
- Cyclophosphamide: Dose in conjunction with nephrology
- Diazoxide: 1-3 mg/kg IV, max. 150 mg, repeat q15min
- Furosemide: 20-80 mg IV; max. 2 mg/kg/d
- Methylprednisolone: 30 mg/kg IV on alternative days for 3 doses, followed by oral prednisone (dose in conjunction with nephrology)
- Nitroprusside: 0.3-10 μg/kg/min IV
- Prednisone: 0.5-2 mg/kg/d
Follow-Up
Disposition
Admission Criteria
- Unstable vital signs
- Oliguria, anuria
- Uremia
- Acute renal failure
- Electrolyte abnormality
- Hypertensive emergency
- CHF
- Infectious cause of GN
Discharge Criteria
Healthy patients with no comorbid illness who present with mild hematuria and proteinuria with:
- Stable vital signs
- No signs of infection
- Otherwise normal lab work
- Close follow-up recommended
Followup Recommendations
All patients with glomerulonephritis should follow-up with nephrology
Pearls and Pitfalls
- Discussion with nephrology if management with immunosuppressives is sought.
- The finding of proteinuria or hematuria should always prompt follow-up to ensure that the patient is not progressing to GN.
Additional Reading
- Balogun RA, Abdel-Rahman EM. Therapeutic plasma exchange and renal related vasculitis: Therapeutic apheresis academy 2010. J Clin Apher. 2011;26:291-296.
- Beck LH Jr, Salant DJ. Glomerular and tubulointerstitial diseases. Prim Care. 2008;35(2):265-296, vi.
- Glassock RJ. Glomerular disease in the elderly.Clin Geriatr Med. 2009;25(3);413-422.
- Glomerular disease. In: Longo D, Fauci A, Kasper D, et al., eds. Harrisons Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill; 2011.
- Walters GD, Willis NS, Craig JC. Interventions for renal vasculitis in adults. A systematic review. BMC Nephrol. 2010;11:12.
See Also (Topic, Algorithm, Electronic Media Element)
- Nephritic Syndrome
- Nephrotic Syndrome
- Renal Failure
Codes
ICD9
- 580.0 Acute glomerulonephritis with lesion of proliferative glomerulonephritis
- 583.4 Nephritis and nephropathy, not specified as acute or chronic, with lesion of rapidly progressive glomerulonephritis
- 583.9 Nephritis and nephropathy, not specified as acute or chronic, with unspecified pathological lesion in kidney
- 583.2 Nephritis and nephropathy, not specified as acute or chronic, with lesion of membranoproliferative glomerulonephritis
ICD10
- N00.9 Acute nephritic syndrome with unsp morphologic changes
- N01.9 Rapidly progr nephritic syndrome w unsp morphologic changes
- N05.9 Unsp nephritic syndrome with unspecified morphologic changes
- N05.5 Unsp nephritic syndrome w diffuse mesangiocap glomrlneph
SNOMED
- 36171008 Glomerulonephritis (disorder)
- 68544003 Acute post-streptococcal glomerulonephritis (disorder)
- 236392004 Rapidly progressive glomerulonephritis (disorder)
- 80321008 Mesangiocapillary glomerulonephritis (disorder)