IgA nephropathy
Description
● Overview:
➣IgA nephropathy is the most prevalent cause of glomerulonephritis globally, and it rarely advances to end-stage renal disease (ESRD) in patients with FSGS.
➣A young man with microscopic haematuria and nephrotic-range proteinuria developed end-stage renal disease (ESRD) and needed dialysis.
● Case Presentation:
➣ Patient: non-medically ill 26-year-old guy
➣Symptoms: exhaustion, vomiting, and bilateral leg and scrotal oedema for two weeks.
➣ No past medical history of fever, sore throat, rash, haematuria, medication usage, or travel.
➣ Family history: hypertension in the mother.
➣ Exam results:
• Hypertensive urgency, BP 210/100 mmHg.
• 3+ pitting oedema (scrotum + legs).
• No organomegaly, rash, or lymphadenopathy.
● Laboratory Results:
➣ Chemistry of Serum
• BUN 60 mg/dl ↑
At 9.85 mg/dl ↑ (after 12.45 mg/dl), creatinine
• Total protein 4.9 g/dl ↓
• Albumin 2.6 g/dl ↓
➣ Haematology
• Chronic disease-related anaemia (Hb 8.2 g/dl ↓) and platelets (71,000 ↓) (later improved)
• Normal WBC
➣ Lipid Panel
Normal values for TG are 113 mg/dl, LDL is 111 mg/dl, and cholesterol is 209 mg/dl.
➣ A urine analysis
• Dipstick: >1000 mg/dl protein, large blood.
10–20 RBCs/HPF under microscopy.
• The nephrotic range for the protein/Cr ratio is 4.26 g/dl.
● Additional tests
• C4 normal, C3 ↓ (72 mg/dl).
• Negative for ANA, anti-GBM, ASO, ANCA, HIV, syphilis, and hepatitis.
Hypogammaglobulinemia with no M-spike is SPEP/UPEP.
• Renal cortical echogenicity is elevated on imaging (USG, CT).
• Renin-aldosterone and metanephrines in the secondary HTN workup are normal.
● Hospital Course:
➣ Oliguria (less than 500 millilitres per day);
➣ Nicardipine was used to reduce blood pressure;
➣ Furosemide was tried; however, there was no change in urine production;
➣ Haemodialysis (HD) was initiated as creatinine increased;
➣ IV iron + ESA was used to treat anaemia. Following BP management, platelets (suspected malignant HTN–related TMA) improved.
●renal biopsy revealed 29 worldwide sclerotic glomeruli under light microscopy (LM).
• Hyalinosis with segmental sclerosis.
• Two crescents, somewhat.
➣ Immunofluorescence (IF):
• Favourable for kappa, lambda, C3, and IgA light chains.
•IgG, IgM, and C1q are negative.
➣The global glomerulosclerosis seen by electron microscopyClassification by Oxford: M1, E1, S1, T2.
➣The final diagnosis was proliferative IgA nephropathy with FSGS and advanced diffuse sclerosing.
●Key Learning Points/Discussion:
➣IgA nephropathy: frequent in the second to third decade; uncommon development to FSGS & ESRD.
➣Pathophysiology: aberrant IgA1 glycosylation; mesangial IgA deposits (particularly lambda light chain), frequently with C3.
Clinical manifestations include proteinuria, microscopic or extensive haematuria, and in extreme situations, nephrotic syndrome.
➣ Poor prognostic indicators include interstitial fibrosis, sclerosis, tubular atrophy, chronic proteinuria, crescents, ↑ creatinine, ↓ GFR, and hypertension.
➣Compared to IgA nephropathy alone, FSGS with IgA nephropathy causes a quicker reduction in GFR.
➣Management:
• If identified prior to ESRD, immunosuppressants (steroid, cyclophosphamide, plasmapheresis) may be beneficial.
•ACE-I/ARB: ideal for controlling blood pressure and proteinuria.
➣ Monitoring: urine sediment, proteinuria, creatinine, and GFR.
➣ In advanced illness, the prognosis is poor; many patients end up requiring dialysis and a transplant.
Protocol
● Patient Issues
➣ Quick Development of End-Stage Renal Disease (ESRD):
• The patient's renal function rapidly declined, which is unusual and suggests a severe course of the illness.[
➣ Coexistence of Two Renal Pathologies:
• IgA nephropathy and FSGS seldom coexist, and when they do, the prognosis is worse than when they do so separately.
➣ Signs and Consequences:
• The patient most likely had oedema, hypertension, proteinuria (protein in urine), and haematuria (blood in urine).
• These symptoms, which are common in glomerular disorders, need to be closely watched.
●Management and Nursing Interventions
➣ Thorough Evaluation
• To identify early indications of hypertension or fluid overload, routinely check vital signs such as blood pressure, heart rate, and respiration rate.
•To measure fluid balance and identify the evolution of oedema, evaluate daily weights and input/output data.
➣ Electrolyte and Fluid Management
• If advised, limit fluid intake to prevent fluid excess and manage oedema.
• Keep an eye on electrolyte levels, particularly potassium and sodium, as hazardous imbalances can result from changes in renal function.
➣ Dietary Changes: Work with a dietitian to establish dietary limitations, like consuming less protein, salt, and potassium. A healthy diet aids in symptom management and problems avoidance.
➣Administration of Medication
•Take steroids, immunosuppressants, or antihypertensives as directed, and keep an eye out for any negative responses or side effects.
• Inform the patient and their family about possible adverse effects and drug adherence.
➣ Patient and Family Education:
•Inform patients about the illness process, the need of follow-up treatment, and the warning signals of increasing symptoms, such as shortness of breath, increased swelling, and reduced urine output.
•Offer information and support resources about the possibility of requiring renal replacement treatment in the future.
➣ Psychosocial care:
•Provide the patient and their family with psychosocial care that addresses the emotional toll that chronic renal disease and the possibility of ESRD progression might take.
•As appropriate, make referrals to support groups or counselling for managing a chronic condition.
➣ Advanced Therapies Preparation:
•If the patient will inevitably develop to end-stage renal disease (ESRD), prepare them for potential dialysis or kidney transplantation by teaching them about access care and what to anticipate during treatments.
Notes
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