LYMPHOCYTIC HYPOPHYSITIS ASSOCIATED WITH DACRYOADENITIS - AN AUTOIMMUNOLOGICALLY MEDIATED SYNDROM
A.M. Joussen1, C. Sommer2, C. Flechtenmacher3, H.E. Voelcker1
The current knowledge about immunological processes is growing. We present a lymphocytic hypophysitis which was followed by a dacryoadenitis.
Case: A22 years old patient first presented in the clinic with a significant swelling of her left lacrimal gland. She reported a history of a hypophysectomy, which was performed transsphenoidally due to enlargement of the gland which was suspicious for a tumor. Histologically a lymphocytic inflammation of the hypophysis was found. The patient was currently on a substitution therapy of the hormons. Laboratory tests as well as ultrasound examination of the lab did not show an signs for lymphoproliferative disease. Rheumatology examination was also unspecifically. There were no laboratory findings concerning infectious diseases. Due to the possible immunological origin we performed a high dose steroid therapy. During this treatment a reduction of the periorbital swelling was visible; the patient remained free of symptoms for more than 2 years. Histologically both hypophysis as well as dacryoadenitis were infiltrated with a lymphocytes, which could be differentiated as CD3+ T-cells and CD20+ B-cells.
Conclusion: Lymphocytic hypophysitis is a rare disease which easily can be mistaken for tumorous proliferation. As combination with rheumatoid arthitis, thyreoiditis or pernicious anemia is frequent, an immunological pathogenesis is likely. This is the first description of a lymphocytic hypophysistis and dacryoadenitis. Therapeutically systemic immunosuppression is necessary. Lymphoproliferative diseases as well as vasculitis have to be excluded.
Augenklinik (1) und Pathologisches Institut (Abteilung für Neuropathologie (2) und allgemeine Pathologie (3)) der Universität Heidelberg, INF 400, D-69120 Heidelberg