22 Oct 2020

9/28/20: IgA Nephropathy

PGY2 Sophia Ghaus presented a case of a 61 y/o F with a history of HTN, DM, HLD and hypothyroidism presents with diffuse 8/10 abdominal pain and HA.  She states that 4 days ago, she developed acute abdominal pain at rest, relieved when lying supine/sideways and associated with fevers, chills, nausea/vomiting.  One week prior to admission, she had traveled to Mexico but denies sick contacts and changes in diet.  5 months prior to admission, the patient noticed worsening abdominal distention and b/l LE edema, along with increasing dyspnea on exertion.  She has difficulty with ADLs because of her increased girth and 20lb weight gain.  She also had decreased urinary frequency, but denies other urinary symptoms.  Patient was born in Mexico and moved to LA several years ago.  Denies any tobacco/drug/alcohol use.

Vitals: T 36.5, HR 126, RR 18, BP 150/87, O2 sat 100% RA

Physical Exam significant for patient appearing somnolent but A&Ox3, +systolic ejection murmur heard throughout, clear lung exam, abdomen distended with shifting dullness but non-tender and 3+ pitting edema in b/l UE and LE.

CBC: WBC 9.3> Hb 10.9/Hct 31.1 <Plt 224 (MCV 97.4, RDW 13.9%)

Diff: 63% PMN, 27% lymph, 8.7% monocytes, 1.7% eosinophils, and 0.9% basophils

CMP:

Na 139/K 3.4/Cl 104/HCO3 21/BUN 33/Cr 2.19 <Glucose 182 (Baseline Cr 0.9, last measured 9 mo ago)

ALP 135>T.protein 6.7/Albumin 2/AST 75/ALT 29/Tbili 0.7/Dbili 0.3

Coags: PT 14.2, INR 1.12

Cardiac workup:

  • Trop <0.01
  • BNP 1265 (normal <125)
  • EKG
  • CXR
  • TTE: EF is 70-75%, Grade 2 diastolic dysfunction.

Infectious workup:

  • HIV negative
  • Acute hepatitis panel nonreactive
  • BCx negative x2

Liver workup:

  • Ammonia 81 umol/L (normal 11-48)
  • Paracentesis:
    • Nucleated cells 81/cumm(1% seg)
    • Albumin 0.2 (Serum Albumin 2)
    • Protein 1.2
    • Culture negative
    • AFB Culture negative
  • C3 69 (normal 90-180)
  • C4 9.1 (normal 10-40)
  • Autoimmune
    • ANA negative, dsDNA negative
    • LKM Ab negative
    • Mitochondrial Ab negative
  • Abd US revealed hepatic cirrhosis, moderate volume ascites, R and L kidneys were unremarkable

Renal workup:

  • VBG: 7.42/32/36/21, FiO2 21%
  • Lactate 2.4
  • UA: >300 protein, large blood (26-50 RBC), negative leuk/nitrites, negative urine eosinophils
  • FeNa 0.2%
  • FeUrea 22.6%
  • Urine protein >1000, UCr 190
  • Utox negative
  • PLA2R negative

Heme workup:

  • Iron panel
    • Iron 65
    • TIBC 114
    • Iron Sat 57%
    • Ferritin 281
  • SPEP/UPEP no monoclonal protein bands
  • K/L: 300/162.4 = 1.85 Inormal <1.65)
  • IgA 962 (normal 70-400)
  • IgG 1660 (normal 700-1600)
  • IgM 63 (normal 40-230)
  • Haptoglobin <10
  • LDH 172

Renal Biopsy was performed which showed IgA nephropathy with membranoprolierative glomerulonephritis pattern.

Treatment: The patient was given lactulose for her somnolence with improvement in mental status on hospital day 2.  She was started on prednisone 30mg BID after renal biopsy resulted in IgA nephropathy and she was discharged with renal and liver clinic follow-up.

Take home points:

  1. Endo Teaching point: patient with volume overload, low voltage EKG, altered mentation and normocytic anemia can be hypothyroid and have myxedematous ascites. In this case, the vital signs and renal findings are not consistent with hypothyroid.
  2. IgA is the most prevalent primary chronic GN worldwide and cirrhosis as the leading cause of secondary IgA.
  3. IgA pathogenesis: Galactose-deficient IgA1 + anti-IgA antibodies form complexes that deposit in the mesangium.
  4. Treatment: ACE-inhibitors (first line), steroids, immunosuppressive medications, cytotoxic agents, plasmapheresis

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