16 Jan 2021

11/30/20: Goodpasture Disease

PGY3 Brent Hiramoto presented a case of a 56 year old male with history of T2DM and HTN presents with persistent cough s/p recent course of Augmentin 2 weeks ago.  The cough is nonproductive, now associated with dyspnea and small volume hemoptysis.  He notes a 10-lb weight loss over the past 2 weeks.  More recently in the past week, he has noted generalized fatigue, ankle swelling, and facial swelling with subjective fevers and chills.  He denies sick contacts, recent travel, incarceration, hematuria, dysuria, abdominal pain, nausea and vomiting.  No chest pain, orthopnea, or PND.  Patient does report non-bloody diarrhea over the past few days and recent decline in urine output.  Home medications include Aspirin 81mg qday, Benazepril 10mg qday, Glimepiride 4mg qday, and Simvastatin 10mg qday.  Social history significant for the patient being born in El Salvador and he moved to LA 18 years ago.  He works in gardening and painting.  Prior heavy alcohol use but quit 10 years ago.  Denies any other drug or tobacco use.

Vitals: T 37.3, HR 80, RR 22, BP 132/62, O2 sat 100% on RA

Physical Exam significant for well appearing male, clear cardiac and pulmonary exam, 1+ b/l LE pitting edema and no rashes/bruising on the skin.

CBC: WBC 11.1> Hb 9.3/Hct 27.3 <Plt 339 (MCV 90.7, RDW 12.9%)

CMP:

Na 134/K 7.2/Cl 109/HCO3 13/BUN 89/Cr 8.48 <Glucose 88

ALP 66>T.protein 6.6/Albumin 3.1/AST 19/ALT 21/Tbili 0.2/Dbili <0.2

Ca 8.2, Mg 2.1, Phos 5.9

Coags: PT 16.4, INR 1.34

CXR showed bilateral patchy confluent airspace opacities throughout both lungs with nodular opacities, trace L pleural effusion:

Infectious workup done with following findings:

  • Negative Flu study, BCx, UA, UCx, Respiratory culture
  • Viral studies negative for CMV, HIV
  • Fungal studies negative for Crypto, Histo, Cocci, Aspergillus and PCP
  • Negative RPR
  • AFB sputum negative x3, MTB PCR negative x2
  • Hepatitis serology negative except for Hep B core Ab was reactive
  • TTE with normal EF and no valvular abnormalities

Renal workup done with the following findings:

  • UA significant for negative leuk/nitrite, large blood (>50 RBC) and 100 protein
  • FeNa 9.1%, FeUrea 69.3%
  • Uprotein/cr: 2.93
  • SPEP/UPEP with no aberrant protein band observed
  • PLA2R negative
  • Cryo negative
  • Anti-GBM IgG Positive

Rheum workup done with the following significant studies:

  • Elevated ESR and CRP at 50 and 29.2 respectively
  • ANA and dsDNA negative, normal complement levels
  • P-ANCA elevated at 1:320 titer

CT Thorax obtained with multifocal peribronchovascular nodular consolidative opacities throughout all lung lobes with cavitation of at least one opacity and more focal pulmonary nodules in a peribronchovascular distribution.

Kidney biopsy obtained which revealed ANCA glomerulonephritis, crescentic subtype with weak linear IgG staining of glomerular basement membrane.

Treatment: Bronchoscopy done with evidence of pneumonia and patient was treated with Levaquin.  Permacath was placed for initiation on HD.  After results from biopsy returned, patient started on steroids, Cytoxan and plasmapheresis.  Patient was discharged with continued renal, pulm, rheum followup.

Teaching points:

  1. Goodpasture disease presents with rapidly progressive glomerulonephritis often accompanied by pulmonary involvement.
    1. Goodpasture syndrome: syndrome of GN + pulmonary hemorrhage
    1. Goodpasture disease: differentiated by anti-GBM mediated disease
  2. Diagnosis: anti-GBM antibodies, presence of glomerulonephritis and/or alveolitis and kidney biopsy demonstrating immunostaining with anti-GBM antibodies
  3. Treatment: Corticosteroids with prolonged taper, Cyclophosphamide 2-3mg/kg/day x2-3 months and 14day course of plasmapheresis or until Ab levels fully suppressed

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