The diagnosis of Autoimmune Hypophysitis (AH) is still mainly a diagnosis of exclusion. Patients often undergo surgery because they are suspected of having a pituitary adenoma. The histological exam, however, shows autoimmune hypophysitis instead. The accurate diagnosis of AH has been greatly facilitated by the introduction of magnetic resonance imaging (MRI). However, it is safe to say that no other pituitary mass lesion so consistently challenges a correct pre-operative diagnosis as AH. Of clear benefit would be the development of a screening test based on the autoimmune pathogenesis of this disease. In most of the patients reported in the literature the diagnosis was based on histology, obtained either after surgery (223) or at autopsy (26), see table below.

We believe, however, that autoimmune hypophysitis is not a surgical disease, and surgery could be avoided if the diagnosis of AH could be established without histology. Surgery should be reserved only for those cases where the pituitary mass seriously endangers the patient's vision.

How Diagnosis Was Made	No. of Cases
Based on histology	249
Surgical pathology	223
Autopsy	26
Based on clinical and radiological criteria	76
Based on clinical criteria only	7

After the diagnosis
Because of the only recent improvement in understanding of autoimmune hypophysitis, a small number of cases have been identified before a surgery approach. For this reason it is difficult to suggest a flow-chart of therapy, prognosis and follow-up.

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• The Primary Care Provider

  If you think you are affected by Autoimmune Hypophysitis, see your primary care provider. they will be able to determine if your symptoms are related to this disease. A good history and a general examination are usually sufficient to make a diagnosis. Asking you about the symptoms, their eventual relation with a pregnancy and, your positive history for other autoimmune diseases, they can suggest exams you can take to confirm the diagnosis. He can also identify the best kind of specialist you need. At this time, only general examination can be performed, testing inflammatory parameters (VES, PCR, and others) or general function of vital organs (basic hematological assays).

• The Endocrinologist

  The endocrinologist is the specialist who can best understand whether your symptoms (bitemporal hemianopsia and headache for example) may be related to a pituitary problem. A physical examination, with more attention to endocrine glands and their targets, such as breast and thyroid, can confirm this diagnosis. The endocrinologist can also suggest which kind of hormonal examination you should have, to check if your pituitary is working properly. At this time only the basic functions of thyroid, adrenal, gonads and eventually GH (in children) need to be checked. Finally, in concert with your medical practitioner, your endocrinologist can identify some autoimmune markers (other organ-specific autoantibodies), that could be positive, especially when autoimmune hypophysitis is associated with other autoimmune disease.
  
  When the diagnosis has been confirmed you need to come back to your endocrinologist, to decide if it is necessary to completely test the pituitary function, if you need a specific therapy or if you need follow-up only.

• The Ophthalmologist

  Your ophthalmologist is the best doctor to diagnose bitemporal emianopsia. They can differentiate between the visual defects deriving from pituitary compression on the optic chiasma, and those caused by retinal problems, which are not related with pituitary enlargement. The field of vision examination is a easy exam which tests the sensitivity of your optic tract. When the optic chiasma is compressed in the middle, the visual field is defective in its peripheral parts: the retinal sensitivity decreases because of the compressive effect of the gland enlargement.

• The Radiologist

  It is best if your primary care provider, together with your endocrinologist and your ophthalmologist, talk together to decide the strategy in choosing the best imaging approach. Usually, all of them, agree on the fact that MRI represents the most sensitive and specific way to confirm a pituitary enlargement. Speaking with the radiologist, will help your primary care provider to be more aware of radiological signs that can predict an autoimmune disease. In fact, the role of the radiologist is the most difficult, because sometimes he can not differentiate between an inflammatory enlargement or a pituitary tumor.

After the diagnosis
Because of the only recent improvement in understanding of autoimmune hypophysitis, a small number of cases have been identified before a surgery approach. For this reason it is difficult to suggest a flow-chart of therapy, prognosis and follow-up.

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• Therapy

  Sometimes autoimmune hypophysitis tends to recover spontaneously without any treatment. There is no absolute agreement among endocrinologists about the best therapy. Because of the autoimmune-inflammatory nature of the pituitary enlargement, corticosteroids are the most common medication used. The goal of the therapy is first to decrease the gland and relieve the compression on the dura mater and optic chiasma. Corticosteroids have also immunosuppressive effect, and their use can reduce the autoimmune reaction in the gland. Because a persistent damage (and the consequent functional deficiency) depends on the percentage of normal tissue that has been substituted by fibrosis, the therapy needs to be begun as fast as possible.

• Prognosis

  The prognosis is quite variable. In fact a wide number of variables can interfere with that: the grade of autoimmune infiltration in the gland, the percentage of fibrosis, the correct response to corticosteroids or the local compressive effects. The prognosis is usually good: the visual defect tends to recover when the gland decreases in size and also headache disappears at that time. Functional impairments are more frequent than persistent visual defects, but fortunately they can be treated by hormone replacement therapy. Levo-thyroxine is used to replace the thyroid function, glucocorticoid and mineralcorticoid are available to treat the adrenal insufficiency and recombinant GH is today obtainable to replace the somatotrophs function. Different problems can happen when the gonadotrophs cells are affected. In fact, although commercial androgens and female hormones are available, they are not capable to treat completely the gonadal insufficiency. In fact, estrogen and progesterone (HRT), alone or in concert with alendronates (or raloxifene in those woman in which estrogen is not indicated), are useful medications for preventing loss of bone mass in menopausal woman, but they does not recover the infertility in young woman. Androgen in different preparation, are also available to treat males affected by impotence derived from gonadotrophs insufficiency. Although steroids are commonly in use, sometimes men refer difficulties in sexual relations, and some of them do not find again fertility.

• Follow-Up

  After the therapy has been posed, the disease needs to be followed-up. The timing and the way vary by case. For example, in those subjects with a complete (anatomical and functional) recover it is necessary only a periodical endocrinological examination. If the recover is partial, the patients needs to be enrolled in a specific program to check the pituitary function (by hormone measurements for example) and the local situation (by imaging or optician examination). In some patients, especially in those with a history of autoimmune diseases, we suggest also to perform a general autoimmune evaluation, checking the most common markers of autoimmune disease.