fibrillary glomerulonephritis
Description
● Overview:
•FGN, or fibrillary glomerulonephritis
About 1% of kidney biopsies are rare.
• Usually sixty years later.
• Characterised by glomeruli on EM that have haphazardly orientated fibrils (10–30 nm).
In contrast to amyloidosis, it is Congo red-negative.
• Strongly stains for C3 and IgG.
• Among Caucasians, more prevalent than among African Americans (8.3:1).
•Linked to autoimmune disorders, multiple myeloma, and HCV.
• The prognosis is poor; ESRD risk is considerable.
➣This case is unique: a 63-year-old male African American was diagnosed with FGN after first being suspected of having hepatorenal syndrome (caused by cirrhosis).
●Discussions:
➣FGN:
• Congo red-negative, distinct fibrils (20 nm).
• Distinct from microtubules, which are immunotactoid GN.
• Renal insufficiency is prevalent, along with proteinuria (typically nephrotic range).
• Polyclonal IgG, C3; often IgG4 dominant immunofluorescence.
•Associations: cancer (especially solid tumours, occasionally haematologic), autoimmune disease, and hepatitis C.
•In this instance, smouldering myeloma with paraproteinemia.
➣ Therapy:
• No curative measures.
• RAS blockade is advised; partial remission is possible.
• The effects of immunosuppressants (cyclophosphamide, MMF, steroids) are not always constant.
• Poor prognosis = around 50% progression to ESRD within 4 years.
•Sclerosis/fibrosis, elevated creatinine, proteinuria, MPGN pattern, and advanced age are all indicators of a bad result.
➣A unique feature of this case is the presence of an African American guy with cirrhosis and a history of HCV.
•FGN is linked to smouldering myeloma instead of active illness.
•emphasises how crucial a biopsy is in differentiating cirrhosis with renal failure from HRS.
●Results of Kidney Biopsies
➣Light Microscopy:
•12 glomeruli: thicker and duplicated capillary membranes, nodular sclerosis, and progressive mesangial expansion.
• No inflammation, necrosis, or crescents.
• Mild arteriole thickening and focal interstitial fibrosis.
• Negative Congo red.
➣ Immunofluorescence:
• In glomeruli, there is strong diffuse IgG (2–3+) and C3 (2+).
•IgA, IgM, and C1q are negative.
• Shows good results for both lambda and kappa.
Electron microscopy:
• Effacement of podocyte foot processes.
• In mesangial and capillary loops, coarse, randomly distributed fibrils (18–25 nm) are present.
• Differs from amyloid (smaller fibrils, positive for Congo red).
●First Workup and Administration:
➣ Paracentesis with a large volume (8.5 L removed).
➣Analysis of fractional excretion: prerenal pattern (FeUrea 5.5%, FeNa <1%).
➣Although proteinuria (2.3 g/day) surpassed HRS limits (<500 mg/day), the albumin challenge showed no improvement, raising the possibility of hepatorenal syndrome.
➣Autoimmune panel: negative for other autoantibodies; +anti-Smith Ab.
Verify cirrhosis using MRI, iron correction, and repeat paracentesis (7.2 L).
Protocol
● Problems Faced
Proteinuria and Oedema: Patients usually have swelling from fluid retention and a considerable loss of protein in their urine.
➣Hypertension: Kidney impairment increases the risk of high blood pressure.
➣Renal Dysfunction: A progressive decline in kidney function that may result in end-stage renal failure or chronic kidney disease.
➣Weakness and Fatigue: Widespread signs of the body's inability to keep fluid and electrolyte balance.
➣Psychosocial Impact: Anxiety, despair, and a worse quality of life can result from chronic illness and uncertainty.
●Nursing Management and Interventions
➣Observation and Evaluation:
• To identify worsening oedema or fluid overload, vital signs, particularly blood pressure and daily weight, should be regularly and thoroughly monitored.
•To track kidney function, strict I/O charting is used.
• Regular monitoring for indications of problems, such as infection or electrolyte imbalance.
➣Diet and Fluid Management:
• Adhere to the recommended fluid limitation.
•Work with dietitians to develop a kidney-function-appropriate low-protein, low-sodium diet.
•If readings are high, teach people to avoid foods rich in phosphorus or potassium.
➣Medication Administration:
• As directed, administer immunosuppressive drugs, diuretics, or antihypertensives.
· Keep an eye out for bad drug reactions and report them as soon as possible.
Notes
for more details visit 10.7759/cureus.28250
