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Habibagahi Z, Ruzbeh J, Yarmohammadi V, et al. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus. Iran J Med Sci. 2015;40(4):372-5
Habibagahi, Z., Ruzbeh, J., Yarmohammadi, V., Kamali, M., & Rastegar, M. H. (2015). Refractory Angioedema in a Patient with Systemic Lupus Erythematosus. Iranian journal of medical sciences, 40(4), 372-5.
Habibagahi, Zahra, et al. "Refractory Angioedema in a Patient with Systemic Lupus Erythematosus." Iranian journal of medical sciences vol. 40,4 (2015): 372-5.
Habibagahi Z, Ruzbeh J, Yarmohammadi V, Kamali M, Rastegar MH. Refractory Angioedema in a Patient with Systemic Lupus Erythematosus. Iran J Med Sci. 2015 Jul;40(4):372-5. PMID: 26170526; PMCID: PMC4487465.
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Iran J Med Sci. 2015 Jul;40(4):372-5.

Refractory Angioedema in a Patient with Systemic Lupus Erythematosus.

Iranian journal of medical sciences

Zahra Habibagahi, Jamshid Ruzbeh, Vahide Yarmohammadi, Malihe Kamali, Mohammad Hassan Rastegar

Affiliations

  1. Department of Rheumatology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran; ; Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
  2. Department of Nephrology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran; ; Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
  3. Department of Internal Medicine, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

PMID: 26170526 PMCID: PMC4487465

Abstract

Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported. In lupus patients, angioedema may be the result of an acquired type of C1 inhibitor deficiency, most probably due to antibody formation directed against the C1 inhibitor molecule. Herein we report a new case of lupus nephritis that developed angioedema and a rapid course of disease progression with acute renal failure and alveolar hemorrhage without response to high dose steroid and plasmapheresis.

Keywords: Angioedema; Enalapril; Systemic lupus erythematosus

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