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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]
  • We also review the published literature on the presenting symptoms, time to presentation, investigations, imaging, treatment and follow-up of ipilimumab-induced autoimmune lymphocytic hypophysitis.[ncbi.nlm.nih.gov]
  • Autoimmune hypophysitis is of particular importance because its presentation can be subtle but life threatening.[ncbi.nlm.nih.gov]
  • In this article, we present the case of a 58-year-old female patient who presented to the emergency room with gradually worsening nonspecific symptoms of headache, nausea, vomiting and decreased oral intake of one week duration.[ncbi.nlm.nih.gov]
  • To aid in this distinction, the authors recently published a clinicoradiological scoring system, which they used in the case of a 15-year-old girl presented here.[ncbi.nlm.nih.gov]
Burning Pain
  • Last year I also started feeling this burning pain in my liver area. I underwent lots of tests and doctors continued to tell me they couldn't find anything wrong. I am amazed to find this and others with the condition saying they have the same pain![medhelp.org]
HELLP Syndrome
  • I have a history of severe HELLP Syndrome during the birth of my child 5 yrs ago and wasn't expected to live. Haven't been the same since.[medhelp.org]
Relapsing Polychondritis
  • Others such as birdshot retinochoroidopathy, autoimmune hypoparathyroidism, and relapsing polychondritis are true rarities.[doi.org]
Withdrawn
  • After failure to respond to therapy, active treatment was withdrawn. Methods: The post-mortem findings are reported, with emphasis on the pituitary histopathology, including immunohistochemical elucidation of the inflammatory infiltrate.[journals.lww.com]
Urinary Incontinence
  • An 87-year-old man was admitted with fatigue, anorexia, vomiting, urinary incontinence, and a depressive state.[ncbi.nlm.nih.gov]
Oligomenorrhea
  • After an uneventful third delivery, 24 years before admission, she could not lactate and developed oligomenorrhea and then amenorrhea. Laboratory evaluation revealed panhypopituitarism and pituitary cell antibodies were positive.[ncbi.nlm.nih.gov]
Headache
  • Four years later, severe headache and a MRI suggestive of pituitary adenoma recurred. A relapse of the autoimmune phenomenon seemed the most feasible hypothesis.[ncbi.nlm.nih.gov]
  • […] and menstrual disorders with headache and dizziness.[ncbi.nlm.nih.gov]
  • In this article, we present the case of a 58-year-old female patient who presented to the emergency room with gradually worsening nonspecific symptoms of headache, nausea, vomiting and decreased oral intake of one week duration.[ncbi.nlm.nih.gov]
  • The patient presented with headache, visual field defects, polydipsia, and polyuria, and she was found to have secondary hypogonadism and hypoadrenalism. Magnetic resonance imaging showed a pituitary mass of approximately 2 cm in diameter.[ncbi.nlm.nih.gov]
  • A 67-year-old woman presented with severe fatigue, nausea and headaches following the third dose of ipilimumab, which was being given as treatment for metastatic melanoma (four administrations at three-weekly intervals).[ncbi.nlm.nih.gov]
Frontal Headache
  • A 46-year-old man presented with frontal headache, a visual field defect and general fatigue.[ncbi.nlm.nih.gov]
  • In very large tumors, ophthalmoplegia as well as frontal headaches are common non endocrine symptoms due to lateral extension of the tumor and pressure on the 3rd, 4th and 6th nerves in the wall of the cavernous sinus.[web.archive.org]

Workup

  • Patient’s workup for possible secondary etiologies of hypopituitarism was negative.[jcdr.net]
HLA-DR5
  • HLA DR4 allele, HLA DR5 allele (DR4 DR5) Recent association found with HLA DQ8 and DR53 alleles as well Can be associated with antibody to PIT1 (POU1F1) Risk Factors Female gender Pregnancy Pregnant type 1 diabetics are at even higher risk.[unboundmedicine.com]

Treatment

  • 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]
  • 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]
  • It is licensed for the treatment of unresectable or metastatic malignant melanoma, while multiple clinical trials using this medication in the treatment of other malignancies are ongoing.[ncbi.nlm.nih.gov]
  • We report on a case of autoimmune hypophysitis with severe clinical symptoms that resolved rapidly after treatment with steroids.[ncbi.nlm.nih.gov]
  • Autoimmune hypophysitis has been described in patients on ipilimumab, a humanised monoclonal antibody increasingly used in the treatment of metastatic melanoma.[ncbi.nlm.nih.gov]

Prognosis

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

Etiology

  • As autoimmune etiologies were likely involved in the disorders, autoimmune hypophysitis associated with Graves' disease was arrived at as the plausible diagnosis.[ncbi.nlm.nih.gov]
  • 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]
  • CONCLUSION: Infundibuloneurohypophysitis should be taken into account in the etiological diagnosis of hypopituitarism, particularly if it is associated with diabetes insipidus and in cases of growth hormone deficit, secondary hypogonadism, or multiple[ncbi.nlm.nih.gov]
  • 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]

Epidemiology

  • 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]
  • 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]
  • 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]
  • Growth Regul 4 ( Suppl 1 ): 11 – 19 25 1974 Epidemiology . Philadelphia: W. B. Saunders; 237 – 263 26 1993 A computer program for non-parametric receiver operating characteristic analysis.[doi.org]
  • From retrospective epidemiological studies patients with NFPAs who became hypopituitary do appear to have an increased mortality risk from cerebrovascular events.[web.archive.org]
Sex distribution
Age distribution

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]
  • The detailed pathophysiology, clinical picture, and therapy of patients with pituitary adenomas and hormone hypersecretion are discussed elsewhere and will not be discussed in this chapter.[web.archive.org]

Prevention

  • 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]
  • 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]
  • 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]
  • ., ipilimumab or tremelimumab) General Prevention None Commonly Associated Conditions Aseptic meningitis: unknown if simple association or causal Other autoimmune polyendocrinopathies are the following: Hashimoto thyroiditis; Graves disease is most common[unboundmedicine.com]
  • The question whether early therapy with l-thyroxin in our patient might have prevented the evolution towards carcinoma will remain unanswered.[ijcasereportsandimages.com]

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