Autoimmune hepatitis during intravenous glucocorticoid pulse therapy for Graves' ophthalmopathy treated successfully with glucocorticoids themselves

We report a case of acute hepatitis of autoimmune origin which occurred in a 43-yr-old woman during iv glucocorticoid (GC) pulse therapy for Graves' ophthalmopathy (GO). Prior to therapy, liver function tests were normal with no previous history of liver disorders or conditions predisposing to GC-associated liver damage. After the administration of a 4.7-g cumulative dose of methylprednisolone acetate, there was a marked increase of liver enzymes, prompting immediate discontinuation of iv GC. Nevertheless, liver enzymes increased further, reaching a peak 45 days later, with values 30- to 50-fold greater than those prior to therapy, associated with evidence of impaired liver function. Liver biopsy showed a marked lymphocytic infiltration, likely indicating an autoimmune hepatitis. Based on the assumption that following GC-induced immune suppression, autoimmune hepatitis might have been precipitated by sudden re-activation of the immune system during interpulse periods, we treated the patient with im and then oral GC, in order to re-induce immune suppression. Within three days from re-institution of GC therapy, there was a marked reduction of liver enzymes and amelioration of liver function. Complete normalization was achieved two months later, while the patient was still receiving a low maintenance dose of oral prednisone.     

自身免疫性垂体炎的研究进展

自身免疫性垂体炎是一种罕见的内分泌疾病，多发生于妊娠或产后。其发病机制不明，临床表现多变，主要包括垂体压迫、腺垂体功能减退症、尿崩症和高催乳素血症等相关症状。治疗目的主要是减小垂体肿块的大小和（或）替代有缺陷的内分泌功能。多数患者需要长期使用激素替代治疗。     

Autoimmune hypophysitis

Autoimmune (lymphocytic) hypophysitis is a rare disease that should be considered in the differential diagnosis of any nonsecreting pituitary mass, especially when occurring during pregnancy or postpartum. We have analyzed 370 articles published from January 1962 to October 2004 and identified a total of 379 patients with primary lymphocytic hypophysitis. The present review synthesizes the clinical and research data reported in this body of scientific literature.     

Autoimmune hypophysitis.

Autoimmune (lymphocytic) hypophysitis has emerged as a distinct and specific clinical and pathological disease entity. Although relatively rare compared with other autoimmune endocrine diseases, nearly a hundred cases have been described. The condition is much more common in females (9:1) and appears to have a particular predilection for the pregnant and postpartum states. The anterior pituitary, and less often the neurohypophysis, appear to be the target for inflammatory autoimmune destruction. During the evolution of the disease process, pituitary hyperfunction (usually hyperprolactinemia) has been noted. This disease should now be included in the differential diagnosis of pituitary disorders, especially in females presenting with pituitary enlargement, particularly if symptoms occur in temporal relationship to pregnancy. The disease may form part of the spectrum of the polyglandular autoimmune endocrine disorders. (Trends Endocrinol Metab 1997;8:74-80). (c) 1997, Elsevier Science Inc.     

自身免疫性垂体炎:一种易被漏诊的疾病

自身免疫性垂体炎虽相对少见,却可造成严重的健康问题,大约有一半自身免疫性垂体炎的患者被误诊为垂体腺瘤而接受了不必要的手术.提高垂体自身免疫性垂体炎的诊断率.除了需要加强对该病的认识,积累更多的诊治经验外,还需深入研究和确认垂体的致病抗原,发展血清学试验以用于垂体占位病变的鉴别诊断;其次需要建立垂体自身免疫病患者的登记数据库,为进行基础和临床创新性研究提供病例来源.     

糖皮质激素成功治疗原发性肉芽肿性垂体炎一例报道并文献分析

原发性肉芽肿性垂体炎( primary granulomatous hypophysitis,PGH)是垂体炎的一种,极其罕见,约占手术确诊蝶鞍病变的1％[1],以弥漫分布多核巨细胞为特征,临床表现为垂体功能减退和蝶鞍占位所致的症状,明确诊断需要病理学检查.大多数报道对糖皮质激素治疗无明显疗效[2,3],本文报道1例经手术活检病理确诊的PGH患者用糖皮质激素成功治疗前后的激素和垂体影像学变化,并结合文献探讨PGH的诊断和治疗.     

Sitaxsentan-induced acute severe hepatitis treated with glucocorticoid therapy

Endothelin receptor antagonists are commonly used in the treatment of pulmonary hypertension. Sitaxsentan, a selective endothelin A receptor blocker, induces a mild transaminitis in approximately 3% to 5% of patients, but rarely an acute severe hepatitis. A case involving a 61-year-old female with sitaxsentan-induced acute severe liver failure is presented. Depite withdrawal of therapy, her liver tests failed to improve. After six weeks of monitoring, the patient was administered high-dose corticosteroids, with a good clinical and biochemical response. While endothelin receptor antagonists are postulated to cause hepatitis by inhibition of a bile salt transporter pump, an immune-mediated or idiosyncratic mechanism should be considered.     

Autoimmune hepatitis associated with thyroiditis and hypophysitis. A case report

Autoimmune hepatitis primarily affects women and 40% of cases are associated with extrahepatic autoimmune dysfunction. Thyroiditis, ulcerative colitis and rheumatoid arthritis are the most commonly implicated entities. We present a 46-year-old woman with type-II autoimmune hepatitis and Graves disease who presented deterioration in level of consciousness, her symptoms mimicking severe liver failure. Hormone studies and nuclear magnetic resonance imaging revealed hypophysitis, which led to hypothyroidism and metabolic encephalopathy. The syndrome was resolved with hormone replacement therapy.     

Autoimmune polyendocrine syndrome type 2 associated with autoimmune hypophysitis and coeliac disease

Autoimmune polyendocrine syndromes (APS) are organ-specific autoimmune disorders affecting multiple endocrine glands; these are gradually destroyed by action of autoantibodies. Similarly to other autoimmune diseases, the presence of certain genetic predisposition is an essential prerequisite to the disease development; polymorphism of the main histocompatible system (HLA in humans) appears to play the most important role. APS are categorized into four types, based on what combination of endocrine glands is affected. APS type 1, characterised by hypoparathyreosis, mucocutaneous candidiasis and Addison's disease, is frequently seen in childhood. For a more common APS type 2 to be diagnosed, Addison's disease together with autoimmune thyroiditis (Schmidt's syndrome) and/or together with diabetes mellitus type I (Carpenter's syndrome) must be present. The third type of autoimmune polyendocrine syndromes (APS type 3) involves the same disorder of endocrine glands as type 2 but usually without any defect of adrenal cortex. If the autoimmune endocrine gland disorder does not fulfil the criteria of APS 1-3, the disease may be categorized as autoimmune polyendocrine syndrome type 4. The authors present a case of 33 years old APS type 2 patient who, over 20 years, developed a wide range of autoimmune endocrinopathies, including endocrinopathies that are less common, such as adenohypophysitis, and are associated with other organ-specific diseases (coeliac disease). The case is presented to demonstrate the fact that APS represent a dynamic process and that it is always important to keep in mind that, over time, a patient may develop other autoimmune diseases. To conclude, the authors emphasise the recommendation to test patients with monoglandular endocrinopathy for the presence of any secondary endocrine disorders.     

Autoimmune neutropenia successfully treated with cyclosporine

A case history is presented concerning a 59-year-old female patient with chronic autoimmune neutropenia complicated by recurrent skin infections, mucositis, and conjunctivitis. For subjective reasons she refused treatment with prednisone, but eventually cyclosporine led to an important clinical improvement and an increase of the peripheral granulocyte count. Treatment modalities of autoimmune neutropenia are briefly discussed.     

Glucocorticoid-induced diabetes mellitus in patients with systemic lupus erythematosus treated with high-dose glucocorticoid therapy

The aim of this study was to investigate the prevalence and associated factors of glucocorticoid-induced diabetes mellitus (GDM) in patients with systemic lupus erythematosus (SLE) receiving high-dose glucocorticoid therapy. Patients with SLE who had received high-dose glucocorticoid therapy (prednisolone ≥1?mg/kg/day) at Yonsei University Medical Center, Seoul, Korea, were recruited between January 1999 and June 2009. In total 127 patients with SLE were evaluated. Sixteen (12.6%) of them developed GDM after high-dose glucocorticoid therapy (95% confidence interval, 6.8-18.4%). Univariate analysis showed that old age, family history of diabetes mellitus (DM), hypertension, higher body mass index, higher mean dose of prednisolone before high-dose glucocorticoid therapy, and concurrent use of mycophenolate mofetil (MMF) were factors that would increase the likelihood of GDM. Multivariate analysis determined that age, family history of DM, mean dose of prednisolone before high-dose glucocorticoid therapy and concurrent use of MMF were independent associated factors for GDM. In summary, GDM was developed among 12.6% of patients with SLE after high-dose glucocorticoid therapy. Old age, family history of DM, higher mean dose of prednisolone before high-dose glucocorticoid therapy and concurrent use of MMF were determined to be factors responsible for increasing the risk of developing GDM.     

Acute spinal cord ischemia during aortography treated with intravenous thrombolytic therapy

Acute anterior spinal cord ischemia is a rare but disastrous complication of endovascular aortic procedures. Although intravenous thrombolysis with recombinant tissue plasminogen activator is an effective treatment for acute brain ischemia, its use for the treatment of spinal cord ischemia has not previously been reported. We report the case of a patient who developed anterior spinal cord ischemia during diagnostic aortography He was treated with intravenous recombinant tissue plasminogen activator within 3 hours after the onset of symptoms. The patient had a rapid neurologic improvement and was discharged from the hospital 3 days after thrombolysis, regaining his ability to walk unassisted. We propose that acute spinal cord ischemia can be treated with intravenous recombinant tissue plasminogen activator within 3 hours after the onset of symptoms, as can any other case of acute ischemic stroke.     

Autoimmune pancreatitis successfully treated with ursodeoxycholic acid

A 51-year-old woman with autoimmune pancreatitis is reported in whom treatment with ursodeoxycholic acid (UDCA) was beneficial. Complaining of epigastric discomfort, she presented with liver dysfunction of the cholestatic type, and diabetes mellitus. Pancreatic imaging revealed a diffuse swelling of the body, an irregular narrowing of the main pancreatic duct, and a terminal stricture of the common bile duct. Histologically, the biopsied pancreas was replaced by fibrous tissue with a small amount of mononuclear cell infiltration. She had anti-carbonic anhydrase-II antibody and anti-lactoferrin antibody. After treatment with UDCA, her liver dysfunction and diabetes mellitus improved and the pancreas size was reduced. Steroid therapy is usually indicated for this disorder, but UDCA may be given as an alternative choice.     

New-onset acute heart failure after intravenous glucocorticoid pulse therapy in a patient with Graves' ophthalmopathy

A 53-yr-old previously healthy man was admitted to our hospital for thyrotoxicosis without ophthalmopathy. Initial therapy with propylthiouracil caused an acute elevation of liver enzymes. Then, he received a first course of ¹³¹I therapy (20 mCi). At the end of 6-mo follow-up after ¹³¹I, he was still thyrotoxic and developed moderately severe ophthalmopathy. The patient refused thyroid surgery and decided to undergo second course of ¹³¹I therapy (30 mCi). Concomitantly with the ¹³¹I, we opted to give high-dose pulse glucocorticoid therapy (PGT) to prevent further deterioration of GO. The patient was started on intravenous methylprednisolone pulse therapy 1 g daily in a cycle (one cycle every 2 wk, each cycle comprising two infusions on alternate days). After the end of the second day of PGT administration, he suddenly developed onset of acute pulmonary edema and hypertension. There was no previous history of cardiac disorder or conditions predisposing to cardiac failure other than thyrotoxicosis. A presumptive diagnosis of fluid overload and/or hypertension-induced acute heart failure was made. After prompt investigations excluding cardiogenic causes, we thought that this condition was triggered by PGT that was superimposed on thyrotoxicosis-related hemodynamic instability. Graves' patients with uncontrolled thyrotoxicosis should be under careful surveillance when PGT is planned. To our knowledge, this is the first reported case of life-threatening acute pulmonary edema caused by PGT in GO.