SHM Converge Scientific Abstract ePoster Gallery-Acute C3 Glomerulonephropathy While on Immunosuppressive Therapy for Bullous Pemphigoid

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This case report describes a rare coexistence of bullous pemphigoid (BP) and C3 glomerulonephropathy in a 70-year-old woman. BP is an autoimmune blistering disease marked by C3 and IgG deposition in the skin basement membrane, while C3 glomerulonephropathy involves predominantly C3 deposition in the renal glomeruli. Although both conditions involve complement-mediated immune injury, their coexistence has seldom been reported.<br /><br />The patient had chronic BP treated with mycophenolate mofetil and prednisone, along with interstitial lung disease. She had a normal baseline creatinine of 0.4 mg/dL and no prior renal disease. She initially presented with hematuria, chills, and creatinine of 1.25 mg/dL, was discharged on ciprofloxacin, and returned three days later with anuria and severe acute kidney injury (creatinine 8.5 mg/dL). She was treated with pulse methylprednisolone followed by prednisone, but developed uremic symptoms requiring hemodialysis two days later.<br /><br />Workup showed positive ANA and c-ANCA, but testing for anti-GBM, anti-dsDNA, anti-Smith, SSA/SSB, PR3, MPO, phospholipase A2 receptor, hepatitis B/C, HIV, and cryoglobulins was negative. Imaging showed only left renal nephrolithiasis without obstruction. Renal biopsy revealed diffuse necrotizing and crescentic glomerulonephritis with predominant C3 deposition, confirming C3 glomerulonephropathy.<br /><br />Treatment after diagnosis included prednisone 60 mg daily, cyclophosphamide 50 mg daily, and reduced-dose mycophenolate mofetil. She was discharged after 13 days with creatinine 7.14 mg/dL and continued on dialysis.<br /><br />The authors note that BP has been associated with other glomerular diseases in prior reports, especially membranous nephropathy, but this appears to be the first reported case of BP with C3-dominant glomerulonephropathy. The case raises the possibility of a shared pathophysiologic link and highlights the challenge of managing both diseases with immunosuppression.

Asset Subtitle

Thomas Matoska

Meta Tag

Author List Maxwell Hershey, Pinky Jha, Shanna Su, Thomas Matoska

Category Clinical Vignettes

Concept C3 Glomerulopathy

Concept Bullous Pemphigoid

Concept Complement deposition

Concept Crescentic glomerulonephritis

Concept Immunofluorescence

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin

Presenting Author Thomas Matoska

Track Adult

Keywords

bullous pemphigoid

C3 glomerulonephropathy

glomerulonephritis

autoimmune disease

complement deposition

acute kidney injury

crescentic glomerulonephritis

hemodialysis

mycophenolate mofetil

cyclophosphamide

C3 Glomerulopathy

Bullous Pemphigoid

Complement deposition

Crescentic glomerulonephritis

Immunofluorescence