Faculty Collaboration Database

Long-term follow-up of non-systemic lupus erythematosus glomerulonephritis in patients with hereditary angioedema: report of four cases. Am J Kidney Dis 1992 Jun;19(6):526-31

Date

06/01/1992

Pubmed ID

1595700

DOI

10.1016/s0272-6386(12)80830-4

Scopus ID

2-s2.0-0026647424 (requires institutional sign-in at Scopus site)   13 Citations

Abstract

Hereditary angioedema (HAE) is characterized by a deficiency in C1 inhibitor protein (C1 INH) and by clinical symptoms of episodic swelling of subcutaneous or mucosal tissue. It has rarely been reported in association with non-systemic lupus erythematosus (SLE) glomerulonephritis (GN). A recent report of two cases indicates the prognosis to be poor, with both patients progressing to chronic renal failure 8 and 20 years after diagnosis. This report describes the 5-year follow-up of a previously unreported case of an 8-year-old boy with HAE and non-SLE membranoproliferative glomerulonephritis (MPGN). The patient developed macroscopic hematuria, azotemia, and a vasculitic rash. Treatment included prednisone and cyclophosphamide, resulting in clinical improvement. The present report also summarizes the long-term follow-up of three previously reported cases of HAE and non-SLE GN, 25, 16, and 10 years after their initial presentation. Patients monitored for 25 and 16 years had MPGN and normal renal function and received no therapy. The third patient, monitored for 10 years, had segmental MPGN. This patient presented with urinary abnormalities and, after treatment with prednisone, had improvement in her hematuria. None of these four patients developed chronic renal failure. These observations indicate a variable outcome in patients with HAE and non-SLE GN.

Author List

Pan CG, Strife CF, Ward MK, Spitzer RE, McAdams AJ

Author

Cynthia G. Pan MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Aged
Angioedema
Child
Complement C1 Inactivator Proteins
Cyclophosphamide
Female
Follow-Up Studies
Glomerulonephritis, Membranoproliferative
Humans
Male
Prednisone
Prognosis
Time Factors