30 Jun 2022

8/17/20 Granulomatosis with Polyangiitis (GPA)

PGY3 Lydia Chow presented a case of a 60 year old female with reported history of uterine tumor s/p hysterectomy and intestinal mass s/p colostomy/reanastamosis presents with chest pain and SOB x1 day.  Four days prior to admission, patient experience stomach pain and constipation.  She went to urgent care and was given Docusate.  She continued to feel poorly with subjective fever/chills and 1 episode of pink urine so she returned to urgent care and was given Ciprofloxacin which she took.  At 2300 the night prior to admission, patient developed SOB and chest pain.  Chest pain is sharp, midsternal, intermittent with deep breaths, lasting several seconds, non-radiating, non-exertional.  SOB is constant, causing difficulty for her to talk.  Patient also reports new onset b/l leg swelling.  The patient has no other home medications and was born in Mexico and denies any ethanol/tobacco/drug use.  Vital signs significant for T 36.9, HR 88, RR 25, BP 207/91, satting 97% initially on RA then needing 2L NC.  Exam revealed a female in mild distress due to dyspnea while speaking, trace bibasilar crackles and 2+ pitting edema of b/l LE to ankles.

CBC: WBC 15.5 > Hb 10.0/Hct 30.1 <Plt 401


Na 124/K 7.5/Cl 89/HCO3 12/BUN 149/Cr 12.82 <Glucose 91 (*Of note, patient reports blood work done in Mexico 2 months ago with normal values)

ALP 171>T.protein 7.2/Albumin 3.1/AST 35/ALT 30/Tbili 0.4/DBili 0.2

Coags:PT 16.3, INR 1.33, PTT 33.5

CXR with a finding of soft tissue mass in the superior mediastinum which displaces the trachea to the L.

Pro-BNP 13714 pg/mL (normal <125)

TSH 1.29 (normal 0.5-3.50)

Infectious workup:

  • BCx negative x2
  • UCx negative
  • Lactate 1.0
  • HIV negative
  • Acute hepatitis panel negative
  • TTE negative for endocarditis, EF 65-70%

Renal workup:

  • VBG 7.29/31/41/15, FIO2 27%
  • UA with 100 protein, large blood (>50 RBC), neg nitrite, small leuk (11-30 WBC), no casts seen
  • FeNa 11.5%
  • FeUrea 72.8%
  • Urine protein/cr 1.78
  • PTH 60 (normal 15-65)
  • Vitamin D 25OH 23 ng/mL
  • HbA1c 6%

Rheum workup:

  • ESR 107 mm/hr (normal 0-19)
  • CRP 87.6 mg/L (normal 0-7)
  • ANA negative
  • dsDNA negative
  • C3 83 mg/L (normal 90-180)
  • C4 26.2 mg/dL (normal 10-40)
  • C-ANCA 1:320
  • Prot-3 Ab positive
  • MPO Ab undetectable
  • Anti-GBM negative

CT Thorax w/o contrast revealed a enlarged R thyroid lobe with at least one dominant mass extends to the mediastinum and displaces the trachea to the L.  Multiple nonspecific scattered pulmonary opacities.

CT Sinus w/o contrast had mild mucosal thickening of bilateral maxillary sinuses and bilateral frontal sinuses.

Given pulm and renal involvement seen, a renal biopsy was done which showed RPGN.

Treatment: Patient was treated for acute renal failure with insulin/D50, bicarb, albuterol and started on HD in the MICU.  Once ANCA resulted in positivity, Rheum recommended IV solumedrol 16mg Q8H x3 days.  Once the preliminary renal biopsy returned, patient received plasmapharesis x5 days followed by Rituxan.  She was discharged home with a prednisone taper and HD continuation.  Endocrine followed up on the thyroid nodule with FNA showing benign follicular node.

Take home points:

  1. ANCA associated vasculitides (GPA, MPA, RLV, EGPA) all have similar features on renal histology (focal necrotizing, often crescentic, pauci-immune GN).
  2. GPA is a necrotizing granulomatous inflammation usually involving upper or lower respiratory tract, along with renal involvement.  It affects small to medium vessels. Progression of the disease can be slow in terms of months or fast in terms of days.

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