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Autoimmune Hypophysitis
Hypophysitides Autoimmune

Presentation

It typically presents with hypopituitarism and pituitary mass found by MRI. We report here unusual presentations of two females with AH followed by empty sella syndrome. [ncbi.nlm.nih.gov]

Entire Body System

  • Relapsing Polychondritis

    Others such as birdshot retinochoroidopathy, autoimmune hypoparathyroidism, and relapsing polychondritis are true rarities. [doi.org]

Neurologic

  • Headache

    […] and menstrual disorders with headache and dizziness. [ncbi.nlm.nih.gov]

    She had a 4-month history of amenorrhea and had suffered from headache for 2 months. One month prior to admission, she visited a different hospital emergency room for headache, fever, and vomiting. [synapse.koreamed.org]

    Headache score vs pituitary volume Figure. Headache score vs pituitary volume. [slideplayer.com]

Workup

Patient’s workup for possible secondary etiologies of hypopituitarism was negative. [jcdr.net]

Autoimmune workup did not reveal any abnormality with CRP - 1.2 mg/l (normal < 7 mg/l); antinuclear antibodies (< 1:40), and antineutrophilic cytoplasmic antibodies (< 1:16), anti-TPO Ab - 4 IU/l (< 9), RA factor 4 IU/l (< 20), and anti-TTG Ab- 3.54 IU [ijem.in]

Treatment

Treatment and responses of these patients with autoimmune hypophysitis suggest that glucocorticoid therapy combined with azothioprine is effective treatment for recurrent autoimmune hypophysitis. [ncbi.nlm.nih.gov]

Diagnosis and treatment of autoimmune hypophysitis: A short review. [jhu.pure.elsevier.com]

Prognosis

(Gd) can variably enhance, usually homogeneously 7 dural enhancement may be present 8 infundibulum may be thickened 8 T2 hypointensity in parasellar region can be present and may be useful in differentiating from a pituitary adenoma 4 Treatment and prognosis [radiopaedia.org]

Germinomas have a better prognosis than NGGCTs of which chorioncarcinomas have the worst prognosis. [web.archive.org]

The rarity of xanthomatous hypophysitis has limited the understanding of their natural history, pathogenesis, and prognosis. The number of reported cases is also not sufficient to characterize clinical presentation. [synapse.koreamed.org]

The 3 cases in the present study that were administered high-dose methylprednisolone or levothyroxine sodium therapy demonstrated a positive prognosis. [spandidos-publications.com]

Etiology

Secondary hypophysitis includes the cases where a clear etiological agent can be identified (for example, the administration of immunomodulatory drugs like CTLA-4 blocking antibody), the cases where the inflammation of the pituitary is considered a reaction [jhu.pure.elsevier.com]

Abstract Autoimmune hypophysitis, often referred to as lymphocytic hypophysitis, is defined as an inflammatory condition of the pituitary gland of autoimmune etiology that leads to pituitary dysfunction. [ncbi.nlm.nih.gov]

Epidemiology

In this review, we provide an overview of the epidemiology, diagnosis, pathogenesis, treatment, and the role of organ-specific and antipituitary antibodies as potential markers of LYH. [clinsci.org]

Less commonly affects the infundibulum or posterior pituitary, causing diabetes insipidus and hyperprolactinemia Epidemiology Predominantly seen in women: female > male (5:1) Strong association with pregnancy, most frequently occurring in the gestational [unboundmedicine.com]

References 1 2003 The epidemiology of autoimmune diseases. Autoimmun Rev 2 : 119 – 125 2 1997 Epidemiology and estimated population burden of selected autoimmune diseases in the United States. [doi.org]

Rose Departments of Pathology (P.C., P.C.B., N.R.R.), Epidemiology (C.N.), Neurosurgery (A.O.), Neuroradiology (M.G.P.), and Molecular Microbiology and Immunology (P.C., N.R.R.), The Johns Hopkins University, Baltimore, Maryland 21205 Diabetes and Endocrine [slideplayer.com]

Granulomatous hypophysitis, however, differs from lymphocytic hypophysitis in certain epidemiological features, which are mostly associated with pregnancy and autoimmune diseases [ 6 ]. [kjim.org]

Pathophysiology

Undiagnosed cases due to indolent disease Prevalence Uncommon Found in Etiology and Pathophysiology Most commonly caused by an autoimmune infiltration that includes an admixture of T and B lymphocytes with plasma cells Causes extrasellar pituitary enlargement [unboundmedicine.com]

Revisitation of autoimmune hypophysitis: knowledge and uncertainties on pathophysiological and clinical aspects. Bellastella G, Maiorino MI, Bizzarro A, Giugliano D, Esposito K, Bellastella A, De Bellis A. Bellastella G, et al. [pubmed.ncbi.nlm.nih.gov]

Pathophysiology: The occurrence of pituitary inflammation associated with pregnancy is felt to be secondary to the involution of the pituitary gland that takes place during this time (4,7). [medschool.lsuhsc.edu]

16 A recent report identified autoimmune antibodies against GH (growth hormone) and adrenocorticotropic hormone in a patient with IgG4-related hypophysitis. 17 Nonetheless, collecting further evident cases and analysis is required to characterize the pathophysiology [scielo.br]

Prevention

Patients need vigilant monitoring for cardiovascular instability. [5] Regional anesthesia with moderate doses of local anesthetic can to some extent prevent perioperative crisis due to reduction of stress response. [joacp.org]

These cases highlight the importance of early screening and treatment for hypopituitarism in all subjects undergoing treatment with anti-CTLA-4 therapy to prevent a potentially fatal outcome from secondary adrenal insufficiency, a readily treatable disease [ncbi.nlm.nih.gov]

This will make it possible to prevent delays in treatment and to avoid the consequences of hypopituitarism, particularly unreplaced adrenal insufficiency. References [1] P. Leporati, M. Landek-Salgado, I. Lupi, L. Chiovato, P. Caturegli. [elsevier.es]

Personal or family history of autoimmune disease May be associated with viral infections, particularly meningoencephalitis CTLA-4 antibody cancer treatment (e.g., ipilimumab or tremelimumab) General Prevention None Commonly Associated Conditions Aseptic [unboundmedicine.com]

Avoid skin contact with gel sites to prevent testosterone transfer to others, especially pregnant women and children. Women: if premenopausal give oestrogen via transdermal oestradiol patches. [almostadoctor.co.uk]

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