Hypopituitarism Due to Lymphocytic Hypophysitis in a Patient with Retroperitoneal Fibrosis
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732 Alvarez, Cordido, Sacriscin Postgrad Med J: first published as 10.1136/pgmj.73.865.732 on 1 November 1997. Downloaded from Hypopituitarism due to lymphocytic hypophysitis in a patient with retroperitoneal fibrosis A Alvarez, F Cordido, F Sacristan Summary of 130/70 mmHg and body temperature of We present a 78-year-old woman with 37°C. Cardiopulmonary auscultation showed clinical acute hypopituitarism in whom a systolic murmur and bilateral rales. There pathologic findings showed lymphocytic was marked tenderness after percussion of the hypophysitis and retroperitoneal fibrosis. right costovertebral area. Laboratory blood Lymphocytic hypophysitis should be in- tests revealed an erythrocyte count of cluded in the differential diagnosis of 3.1 x 1012/1, haemoglobin 6.0 mmol/l, haema- hypopituitarism at any age. The associa- tocrit 0.267, leucocyte count 8.7 x 109/1, urea tion with idiopathic retroperitoneal fibro- 34.1 mmol/l, creatinine 335.9 mmol/l, potas- sis suggest an autoimmune origin for this sium 5.5 mmol/l, sodium 135 mmol/l and entity. glucose 6.1 mmol/l. The urinary sediment presented abundant white blood cells and Keywords: hypopituitarism, lymphocytic hypophysi- bacteria. Chest X-ray showed a right pleural tis, retroperitoneal fibrosis effusion. Renal sonogram showed bilateral nephrolithiasis, high-grade right obstructive uropathy, and atrophic right kidney. The Hypopituitarism is most frequently due to a initial diagnosis was urinary tract infection pituitary tumour, other common causes being complicating obstructive uropathy. Empiric surgery, pituitary radiation therapy and pitui- antibacterial treatment was prescribed (aztreo- tary apoplexy. Less common causes ofpituitary nam). Her condition worsened with vomiting, insufficiency include empty sella syndrome, progressive decline in mental status and head trauma, internal carotid artery aneurysm hypotension. Physical examination revealed and infiltrative diseases that affect the hypotha- blood pressure of 90/50 mmHg and dehydra- lamus or pituitary.' tion, without focal neurologic deficits. Re- Lymphocytic hypophysitis is being increas- peated blood laboratory tests showed ingly recognised as a cause of hypopituitarism, haemoglobin 5.6 mmol/l, haematocrit 0.251, usually presenting in women during late leucocyte count 7.67 x 109/1, urea 22.2 mmol/ pregnancy or the postpartum period. It is 1, sodium 144 mmol/l, potassium 5.7 mmol/l, characterised by lymphocytic infiltration and glucose 1.9 mmol/l. Urine sodium was destruction of the anterior pituitary gland. 49 mmol/l and urine potassium 57 mmol/l. http://pmj.bmj.com/ Depending on the stage of the disease, the A presumptive diagnosis of acute adrenal gland may be enlarged due to inflammatory insufficiency was established and the patient infiltrate, or small and atrophic, with fibrosis. received high-dose hydrocortisone. Serum Patients may show symptoms of an expanding cortisol (before the steroid therapy) was intrasellar mass or different degrees ofpituitary 179 mmol/l (reference range 138-690 mmol/ dysfunction.2 1). The thyroid-stimulating hormone level was We report on a woman with lymphocytic 1.2 mU/l (reference range 0.25-4 mU/l) and on October 2, 2021 by guest. Protected copyright. Juan Canalejo Hospital, La Corufia, hypophysitis presenting with clinical signs of the free thyroxine was 7 pmol/l (reference Spain acute hypopituitarism and idiopathic retro- range 11 - 24 pmol/l). With these hormonal Department of peritoneal fibrosis, probably of autoimmune results and the highly stressful situation of the Pathology origin. Lymphocytic hypophysitis should be patient, a diagnosis of hypopituitarism was A Alvarez included in the differential diagnosis of hypo- made. No stimulation test could be per- F Sacristan pituitarism at any age. The association with formed. A computed Department of tomographic study did Endocrinology idiopathic retroperitoneal fibrosis suggests an not show evidence of a hypothalamus-pitui- F Cordido autoimmune origin for this entity. tary mass. After an initial clinical and bio- chemical improvement, with normalisation of Department of Case report serum glucose and electrolyte levels, the Ciencias de la Salud patient died seven days later due to broncho- I, University of la A 78-year-old woman was admitted to the aspiration. Corufia, La Corufia, Spain hospital because of right flank pain during the An autopsy performed four hours after death F Cordido previous two weeks and renal failure. The showed a normal brain (1050 g) and an patient had a 10-year history of bilateral atrophic pituitary gland, hard in consistency Correspondence to obstructive uropathy with bilateral lithiasis and white-grey in colour. The thyroid was Augusto Alvarez Garcia, Servicio de Anatomia and secondary renal atrophy. She was allergic small (6.2 g), as were the adrenal glands (joint Patol6gica, Hospital Juan to penicillin and had previous diagnoses of weight 5 g) and ovaries. There was a retro- Canalejo, Las Jubias 84, renal insufficiency and chronic anemia. She 15006 La Coruna, peritoneal fibrous mass with a diameter of Espana had never been pregnant. Physical examina- 5.3 cm, involving iliac vessels, both ureters and Accepted 20 March 1997 tion disclosed general pallor, blood pressure the right kidney hilium. Kidneys were atrophic, Lymphocytic hypophysitis 733 Figure 1 The posterior pituitary gland normal in Causes of secondary retroperitoneal size, showed no altera- fibrosis Postgrad Med J: first published as 10.1136/pgmj.73.865.732 on 1 November 1997. Downloaded from tions (left), while the ante- * drugs: methysergide, methyldopa, ergot rior part (right) is atrophic alkaloids and by dense fibrous tis- * inflammation adjacent to an abdominal sue (arrows) (HE origi- aneurysm nal x 40) * malignant tumours * retroperitoneal injuries: haemorrhage, infection, or radiation z * peri-arteritis nodosa * autoimmune disease 'T ll * tuberculosis te. l * sarcoidosis lA * biliary tract disease * gonorrhoea * ascending lymphangitis from the lower extremities Figure 2 Close view of the anterior pituitary gland with slight oedema and moderate lymphocy- tic infiltration surround- ing (arrows) the residual Summary points parenchyma (HE origi- nal x 400) * lymphocytic hypophysitis should be considered k.... a cause of hypopituitarism at any age * it presents as an intrasellar mass during the last months ofpregnancy or early postpartum, or as hypopituitarism at any age * the pituitary can be enlarged or small in size * the aetiology of lymphocytic hypophysitis is probably autoimmune. Autoimmune pathogenesis is supported by coexistence with idiopathic retroperitoneal fibrosis with pyelo-calyceal dilatation and coraliform lithiasis. The right kidney weighed 40 g and the left 130 g. Other necropsy findings included an increase in the weight of both lungs (600 g each) due to bilateral bronchopneumonia, and Discussion left ventricular hypertrophy due to subaortic stenosis. Hypopituitarism without a pituitary tumour or Microscopically, the posterior pituitary previous pituitary-directed therapy could be http://pmj.bmj.com/ gland was normal. Parenchymal fibrosis was due to pituitary apoplexy, empty sella syn- found in the anterior pituitary gland (figure 1), drome, head trauma, internal carotid artery with slight oedema and a moderate T-pheno- aneurysm and infiltrative diseases affecting the type lymphocytic infiltration (CD3+) , with hypothalamus or the pituitary.' plasmatic cells and few eosinophils (figure 2). Lymphocytic hypophysitis is a rare entity, No germinal centres, giant cells or granulomas with approximately 60 reported cases, predo- were observed. The histologic appearance of minantly affecting females and usually present- on October 2, 2021 by guest. Protected copyright. the fibrous retroperitoneal mass was similar to ing during late pregnancy or the first 14 that of the anterior pituitary gland, with months thereafter.3 It presents as an intrasellar collagen tissue and a scattered infiltrate of T- mass during the last three months ofpregnancy phenotype lymphocytes (CD3+) and polyclo- or early postpartum period, or as postpartum nal plasmatic cells. Isolated germinal centres hypopituitarism ofvariable degree.2 The loss of were observed. Lymphocytic infiltrates and pituitary function is often out of proportion to fibrosis were not observed in the thyroid, the degree of pituitary enlargement. Depend- adrenal glands or ovaries. ing on the phase of the disease, the pituitary gland can be enlarged, normal or small in size. Somatotropic and gonadotropic function are more likely to be preserved than corticotropic Causes of hypopituitarism or thyrotropic function, unlike the findings in hypopituitarism due to a sizeable pituitary * pituitary adenoma adenoma.' It has been reported 4 that anti- * pituitary surgery pituitary antibodies can be found in the serum * pituitary radiation therapy of 75% of women with the empty sella * pituitary aplopexy syndrome. It is conceivable that in some cases * empty sella syndrome * head trauma the empty sella syndrome may represent a final * infiltrative disease stage of lymphocytic hypophysitis.' In addition, * internal carotid artery aneurysm in some cases of the empty sella syndrome, * lymphocytic hypophysitis there is evidence of resolution of pituitary enlargement caused by previously unrecog- 734 Alvarez, Cordido, Sacristain nised hypophysitis.3 The real incidence of this Because of the occasional presence of anti- entity, therefore, may be greater than currently pituitary antibodies, a lymphoid