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Hypophysitis Secondary to Small Vessel ANCA Vasculitis Treated With Rituximab.
Kumar, Nankee K; Rivadeneira, Ana; Banerjee, Shubhasree; Gubbiotti, Maria; Yoshor, Daniel; Jackson, Christina; Kharlip, Julia; White, Caitlin A.
Afiliação
  • Kumar NK; Department of Neurosurgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
  • Rivadeneira A; Division of Endocrinology, Thomas Jefferson University, Philadelphia, Pennsylvania.
  • Banerjee S; Division of Rheumatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
  • Gubbiotti M; Division of Pathology, MD Anderson Cancer Center, Houston, Texas.
  • Yoshor D; Department of Neurosurgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
  • Jackson C; Department of Neurosurgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
  • Kharlip J; Division of Endocrinology, Diabetes, and Metabolism, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
  • White CA; Division of Endocrinology, Diabetes, and Metabolism, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
AACE Clin Case Rep ; 10(2): 55-59, 2024.
Article em En | MEDLINE | ID: mdl-38523853
ABSTRACT
Background/

Objective:

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis is a rare small vessel vasculitis that can cause pituitary hypophysitis. Hypophysitis is difficult to treat, often requiring high doses of glucocorticoids with frequent flaring as glucocorticoids are tapered. We present a case of ANCA vasculitis involving the pituitary gland successfully treated with rituximab. Case Report Fifty-one-year-old woman developed progressive frontal headaches, congestion, and epistaxis. Sinus computed tomography scan showed pituitary enlargement and chronic mucosal disease. Pituitary magnetic resonance imaging (MRI) confirmed a diffusely enlarged pituitary with a thickened pituitary stalk. Serologic evaluation revealed elevated inflammatory markers, positive perinuclear ANCA (p-ANCA), and an elevated serum anti-proteinase 3 (anti-PR3) antibody. The patient underwent pituitary biopsy, which showed adenohypophysitis with dense lymphoplasmacytic infiltration, some arranged perivascularly, compatible with involvement of the pituitary gland by ANCA vasculitis. The patient began rituximab and reported resolution of daily headaches, congestion, and epistaxis. Pituitary MRI scan 6 months after rituximab showed reduction in pituitary gland size and stalk thickening.

Discussion:

ANCA vasculitis is a rare etiology of pituitary hypophysitis, which can present a diagnostic and therapeutic challenge. Pituitary involvement of ANCA vasculitis can be identified through p-ANCA or cytoplasmic ANCA (c-ANCA) and biopsy of the involved tissue. Rituximab, a monoclonal antibody against CD20, has been successfully used to treat ANCA vasculitis and in this case, led to clinical improvements and reduction in the size of the pituitary gland.

Conclusion:

Pituitary biopsy enabled confirmation of ANCA hypophysitis and facilitated treatment with a steroid-sparing agent.
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Texto completo: 1 Base de dados: MEDLINE Idioma: En Revista: AACE Clin Case Rep Ano de publicação: 2024 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Idioma: En Revista: AACE Clin Case Rep Ano de publicação: 2024 Tipo de documento: Article