Causes of secondary hypophysitis

What are the main inflammatory and infectious causes of secondary hypophysitis?

  • Secondary hypophysitis may be caused by an underlying systemic inflammatory disease, usually multivisceral, or an infectious disease that also involves the pituitary gland and/or stalk. The most common etiologies, and their characteristics, are shown in the below table.
  • In addition, some sellar lesions (e.g., Rathke’s cleft cyst, meningioma, and germinomas) can be associated with secondary inflammatory cellular reactions consistent with hypophysitis.
  • Potential infectious etiologies (very rare): Bacterial (most commonly gram-positive bacteria), granulomatous diseases (e.g., tuberculosis [TB], syphilis, brucellosis), fungal infections (aspergillosis, coccidioidomycosis), and Whipple’s disease.
  • Risk factors for pituitary infections: Immunocompromised patients, hematologic/parasellar infections, cavernous sinus thrombosis, and previous pituitary surgery.
  • Clinical presentation: Most commonly presents with mass effects (e.g., headaches, hypopituitarism, vision defects, and diabetes insipidus [DI]), and less commonly (< 30%) with classic features of infection (e.g., fever, leukocytosis, meningismus).
  • Radiographic features: Pituitary abscess and TB may present as cystic lesions with gadolinium ring enhancement, although often are radiographically nondiagnostic.
  • Diagnosis: Usually made during drainage in transsphenoidal surgery (TSS) and follow-up evaluations for infectious etiologies.
  • Treatment: Parental antibiotics are generally recommended for infectious causes, depending on etiology and drug sensitivities.

Secondary Hypophysitis.

SARCOIDOSISGRANULOMATOSIS WITH POLYANGIITISLANGERHANS CELL HISTIOCYTOSIS (LCH)ERDHEIM-CHESTER DISEASE
Age, gender, incidenceYoung adults, African American females, 1:100,00040–60 years of age; M > F = 2:1Children (< 1:200,0000) > AdultsMiddle age (> 50s)
M = F
Clinical presentationMultivisceral (lungs, heart, eyes, skin, sinuses), neurosarcoidosis: 5%, hypopituitarism/DI: 30%Systemic vasculitis (kidneys, lungs, sinuses, otitis media), DIChildren – DI/GHD
Adults – DI (25%) and diffuse disease (bone, skin, lungs)
Multivisceral (osteosclerosis: knees + ankles, heart, kidneys, liver, lungs, spleen, thyroid)
Evaluation (in addition to pituitary hormones)Serum/CSF ACE, CXR, +/− Chest CTc-ANCA, PR3-ANCA, CXR, Chest and abdomen CTCXR, Chest CT, bone scan, FDG-PET, bone marrow aspirationBone scan, Chest and Abdomen CT
Tissue biopsy resultsNoncaseating epithelioid cell granulomasNecrotizing granulomasLangerhans/dendritic cellsNon–Langerhans cell histiocytosis
TreatmentSupraphysiologic prednisone: start 1 mg/kg/day × 2 weeks, then taperSteroids, rituximab, methotrexateFocal versus systemic chemotherapy and steroids, XRTSteroids, chemotherapy, surgery, XRT, vemurafenib

ACE, Angiotensin-converting enzyme; c-ANCA, cytoplasmic anti-neutrophil cytoplasmic antibody; CSF, cerebrospinal fluid; CT, computed tomography; CXR, chest x-ray; DI, diabetes insipidus; FDG-PET, fluorodeoxyglucose positron emission tomography; GHD, growth hormone deficiency; PR3-ANCA, proteinase 3-ANCA; XRT, radiation therapy.

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