Lymphocytic Panhypophysitis: Its Clinical Features in Japanese Cases

Abstract

Lymphocytic hypophysitis is divided into three forms according to the involved tissues, lymphocytic adenohypophysitis, lymphocytic infundibulo-neurohypophysitis, and lymphocytic panhypophysitis (LPH). The term LPH was first proposed by us in 1995, although its entity and pathogenesis still remain controversial. Here we report five cases of LPH, who visited our clinics during 1994 to 2009. All cases were female of 20 to 77 years of age, and one case was associated with pregnancy. They presented with polyuria (n = 4), headache (n = 3), general malaise, polydipsia (n = 2), blunted vision, diplopia, amenorrhea or appetite loss (n = 1). Magnetic resonance imaging showed the pituitary swelling, the thickened stalk, the loss of the T1 hyperintense neurohypophysis (n = 4), or the atrophic pituitary (n = 1). Endocrinological examinations revealed deficiencies of TSH, ADH in all cases, GH, ACTH in three cases, LH, PRL in two cases, and FSH in one case, respectively. The severity of ADH deficiency varied among the cases. Anti-pituitary antibody was not detected in the cases examined. The biopsy of the pituitary lesions was performed except for one case, all of which revealed the diffuse lymphocytic infiltration. These results suggest that LPH is characterized by the female predominance, the atypical patterns of anterior pituitary hormone deficiencies and the variable degrees of diabetes insipidus in Japanese.

Clinical, Endocrine and Imaging Characteristics of Patients with Primary Hypophysitis

“…The severity and rapid onset of LYH manifestations are not usually correlated with the degree of pituitary enlargement, suggesting that direct immune-mediated destruction of hormone secreting cells, rather than compression or architectural distortion, is responsible for its pathogenesis [4,9]. Granulomatous hypophysitis is usually more severe than LYH [32], similar to the single patient in our series who presented with panhypopituitarism and diplopia.…”

“…Specifically, 36 % showed hormonal recovery (partially or complete) and 41 % a radiological response. Recent retrospective trials have described cases of recovery of a number of axes in PH ranging from 43-50 % [16], although they included also cases of granulomatous and xanthomatous hypophysitis [32,39]. Other series have reported worse outcomes probably because of a shorter median follow-up (from 8-28.9 months) with complete recovery in 17 % of cases [6,17], radiological responses in 50 % and hormonal recovery in 19 % of the LYH cases.…”

An Overview of Diagnosis of Primary Autoimmune Hypophysitis in a Prospective Single-Center Experience

“…Recently, an increasing number of clinical studies and patient series have been published [3,5,15,16] . To our knowledge, our paper for the first time describes a series of patients diagnosed with primary autoimmune hypophysitis at a single center in a prospective view.…”

Diagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society

“…In LINH, the neurohypophysis or stalk is enlarged, and the lesion shows gadolinium enhancement [10,11]. Both LAH and LINH findings are observed in LPH [12,13].…”