Where Have All the Eos Gone? Long Time Passing or in the Heart? ANCA-Associated Crescentic Glomerulonephritis Presenting with Eosinophilic Myocarditis
- Glomerular Diseases: Epidemiology and Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Khan, Mohammad Iqbal, Hawaii Residency Programs Inc, Honolulu, Hawaii, United States
- Parekh, Kunal, Queen's Medical Center, Honolulu, Hawaii, United States
- Kim, Lisa, Queen's Medical Center, Honolulu, Hawaii, United States
Eosinophilic granulomatosis with polyangiitis (EGPA) may be diagnosed from other small vessel vasculitides by clinical criteria. Its rare presentation as an acute coronary syndrome in a community setting makes this an unfamiliar entity. New therapies in IL-5 and complement pathways are recent tools to reduce immunosuppression but trials have mixed results or must be used "off-label" for EGPA due to its variable presentation. We present a case of EGPA in which avacopan was initiated for compassionate use.
A 69 year old male with history of asthma presented to his PCP with 3 days of constant left sided chest pain radiating to the back. Transfer to our institution showed cardiac MR consistent with subendocardial infarct and suspicion for eosinophilic myocarditis as peripheral eosinophilia was elevated to 38.7% (absolute eosinophils 2250/mm3). Endomyocardial biopsy was performed showing sheet-like histiocytic cell proliferation with abundant eosinophilic inflammation (Figure 1). Methylprednisolone 500 mg was initiated for presumed eosinophilic myocarditis. Urinalysis was notable for 51-100 rbc and renal biopsy was performed. A diagnosis consistent with pauci-immune necrotising crescentic glomerulonephritis was made but eosinophils were absent. cANCA was positive at 1:320. The patient received rituximab and discharged with steroid taper but was rehospitalized for Klebsiella and Pseudomonas pneumonia and severely reduced eGFR of 9 mL/min/1.73 m2. Cyclophosphamide was initiated with another round of high dose steroids but dialysis was started by week 9 of his original presentation. On day 58, the patient started avacopan 30 mg BID. Although he eventually became dialysis independent he continued to experience complications from infections and entered hospice 8 months after the diagnosis of EGPA.
EGPA may affect multiple organs and therefore requires rapid recognition with aggressive treatment and coordination from specialists. However, complications often occur with sustained immunosuppression as our case demonstrates. The use of new agents for this rare cause of ANCA associated glomerulonephritis must further be studied.