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]