To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.

Invited Speakers

John Danziger
John DanzigerCase Presenter | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported
Michelle Lai
Michelle LaiHepatology Discussant | Guest Participant, Boston, MA, USA | No conflict of interest reported
Stewart Lecker
Stewart LeckerNephrology Discussant | Beth Israel Deaconess Medical Center, Boston, MA, USA | No conflict of interest reported

Case

The patient is a 60-year-old woman who initially presented 6 years ago with acute hepatitis and jaundice. Hepatic biopsy suggested primary sclerosing cholangitis, although the IgG4 was indeterminate.  She was treated with steroids, and then maintenance with azathioprine, and had marked normalization of her liver function. A clinical diagnosis of steroid responsive autoimmune cholangitis was made. Since then, with weight gain on steroids, she developed diabetes and NASH. NASH improved with weight loss.

Due to diabetes, weight gain, and osteoporosis, the steroids were tapered off. A few months later, as her steroids were tapered to very low doses, she developed edema and high-grade proteinuria (5 g/g on spot urine), with stable renal function.

A renal biopsy was performed (see below).

Despite taking 100 mg of azathioprine, 3 mg prednisone, and 50 mg losartan daily, her proteinuria has progressed to 10 grams, and albumin is down to 3.1 mg/dl.

Medical history:
As noted above

Family history:
Not contributory

Medications:
Azathioprine, prednisone, losartan

Physical Exam:
Obese, 2+ lower extremity edema

Laboratory and other data:
Workup for malignancy has included a negative colonoscopy, mammogram, gynecologic exam, CT abdomen/pelvis, CA19-9, and MRCP.

Kidney Pathology

Pathology images pending

Kidney biopsy suggests secondary membranous (based on negative PLA2R, and IgG subtyping), with no evidence of IgG4/plasma cell involvement.

Questions posed & summary of key discussion points

1. Would you consider her membranous nephropathy secondary to her autoimmune liver disease, despite its stability?
2. Would you immune-suppress further than Imuran/prednisone?
3. Given her possible IgG4 hepatic involvement, and exquisite steroid responsiveness, would you try increasing her prednisone?

Author(s) of case summary:

Case summary pending

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