Rare Pediatrics News
Disease Profile
Lymphocytic hypophysitis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
#N/A
Age of onset
#N/A
ICD-10
#N/A
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
LYH; LH; Autoimmune hypophysitis
Categories
Immune System Diseases
Summary
Lymphocytic hypophysitis (LH) is a condition in which the pituitary gland becomes infiltrated by
Symptoms
Other features that occur more rarely and are related to alterations in pituitary secretions may include:[1]
- hypoprolactinemia (deficiency of prolactin)
- subclinical hypopituitarism (decreased production of pituitary
hormones with no symptoms) - ACTH deficiency
- acute adrenal crisis
- hypogonadotropic hypogonadism (usually diagnosed only in males)
- isolated growth hormone (GH) deficiency
Cause
Treatment
Many affected individuals only have headaches as a manifesting symptom, and there have been multiple reports of these individuals improving with glucocorticoids alone.[4] Although the use of glucocorticoids or other anti-inflammatory and immunosuppressive drugs have been suggested as medical treatment, their long-term efficacy still needs to be confirmed. High-dose methylprednisolone pulse therapy (short, intensive administration given at set intervals) seems to be effective in about 30% of treated patients.[1]
Transsphenoidal surgery (accessed "through the nose") to confirm diagnosis and save viable pituitary
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
Hypophysitis Research Center
Johns Hopkins Schools of Medicine & Public Health
Ross Bldg. Room 660
720 Rutland Avenue
Baltimore, MD 21205
Telephone: +1-443-287-4593
Fax: +1-410-614-3548
E-mail: [email protected]
Website: https://pathology.jhu.edu/hypophysitis/ -
The Endocrine Society
8401 Connecticut Avenue, Suite 900
Chevy Chase, MD 20815
Toll-free: 888–363–6274
Telephone: 301–941–0200
Fax: 301–941–0259
E-mail: [email protected]
Website: https://www.endo-society.org -
The Pituitary Foundation
86 Colston Street
Bristol BS1 5BB
United Kingdom
Telephone: 0117 370 1320
E-mail: [email protected]
Website: https://www.pituitary.org.uk/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Lymphocytic hypophysitis. Click on the link to view a sample search on this topic.
References
- Antonio Bellastella, Antonio Bizzarro, Concetta Coronella, Giuseppe Bellastella, Antonio Agostino Sinisi and Annamaria De Bellis. Lymphocytic hypophysitis: a rare or underestimated disease?. European Journal of Endocrinology. 2003; 149:363-376.
- Peter J Snyder, MD. Causes of Hypopituitarism. UpToDate. Waltham, MA: UpToDate; 2012;
- Schreckinger M, Francis T, Rajah G, Jagannathan J, Guthikonda M, Mittal S. Novel strategy to treat a case of recurrent lymphocytic hypophysitis using rituximab. J Neurosurg. June 2012;
- Bernard Corenblum. Pituitary Disease and Pregnancy. Medscape Reference. July 6, 2011; https://emedicine.medscape.com/article/127650-overview#a30. Accessed 10/1/2012.
Rare Pediatrics News