Lege Artis Medicinae

[IgG4-related disease]

ZEHER Margit

APRIL 22, 2011

Lege Artis Medicinae - 2011;21(04)

[IgG-4-related disease is a clinical entity characterised by significant elevation in serum IgG-4 levels, infiltration of IgG-4+ plasma cells into the involved tissues, enhanced fibrosis, and good therapeutic response to corticosteroids. The IgG-4 associated disease mostly affects two organs. The salivary and lacrimal gland enlargement and inflammation is known as Mikulicz’s disease, which had been previously known as a subtype of Sjögren’s syndrome for a long time. The other commonly involved organ is the pancreas, in which a special form of chronic pancreatitis, namely autoimmune pancreatitis develops. IgG-4 associated disease as a separate disease has been suggested by Japanese authors. Previously published data support the common pathogenesis of autoimmune pancreatitis and Mikulicz’s disease. Besides these two manifestations, similar histological lesions and elevated IgG-4 levels have been demonstrated in many other organs. At present, it is not clearly demonstrated whether IgG-4 syndrome is an autoimmune disorder, and we do not know the exact reason of the elevated IgG-4 levels in patients with this syndrome. IgG-4 is a regulatory immunoglobulin, the main function of which is to decelerate immune responses, thus its pathologic role in tissue destruction is difficult to explain. It is not clear either, whether IgG-4 syndrome is indeed a single disease, or only an example of overlapping symptoms of various diseases. On the basis of the characteristic histological lesions in various organs, IgG-4 syndrome is similar to multi-organ diseases, such as sarcoidosis or vasculitis.]

COMMENTS

0 comments

Further articles in this publication

Lege Artis Medicinae

[Cardiovascular risk of non-steroidal antiinflammatory drugs]

FARSANG Csaba

[During the past decade, a number of original publications, reviews and metaanalyses were published on the cardiovascular safety of nonsteroidal antiinflammatory drugs (NSAIDs). As this group of medicines is among the most frequently used ones and many preparations are available over the counter, it seems to be prudent to summarise the most important results on the safety of these drugs, and underline their potentially harmful cardiovascular side effects. Nevertheless, it can also be emphasized that there are substantial differences between different compounds, and the cardiovascular risk does not depend on the ratio of COX-1/COX-2 selectivity. Cardiovascular risk can be increased by all NSAIDs with the possible exception of naproxen.]

Lege Artis Medicinae

[Halogen addition and steroid effect]

NAGY Nikoletta, KEMÉNY Lajos

[Among locally administered anti-inflammatory drugs used in dermatology, steroids are among the most commonly applied ones. In everyday practice, choosing the right local steroid preparation is not easy, since more than 50 different local steroid preparations with at least 30 different active ingredients are available. The choice of the local steroid preparation depends on a number of aspescts. It is recommended to apply local steroid preparations that, besides having a strong effect, also have favourable side effect profiles. Moreover, it is subservient to apply local steroids that penetrate deeply into the skin, but have minimal systemic absorption, therefore do not inhibit significantly the hypothalamicpituitary- adrenal axis if administered locally. These characteristics of local steroid preparations are determined by chemical modifications at various positions on the steroid-frame. In this study, we examined the different types of chemical modifications, and the relationship between halogen addition and the characteristics of steroid preparations. We compared the local steroid preparations using the data of previous clinical trials. Regarding the efficacy, safety, tolerability and the risk/benefit ratio, the halogenated steroid preparations were overall superior to nonhalogeneted ones. Among the halogenated preparations, the fluticasone propionate, the mometasone furoate and clobetasol propionate were proved to be the most suitable locally administered, very potent or super potent ones.]

Lege Artis Medicinae

[Diagnosis of diabetes mellitus, treatment and care of diabetic patients in the adulthood - Novelties in the position statement of the Hungarian Diabetes Association, 2011]

JERMENDY György

[The position statement of the Hungarian Diabetes Association has been renewed in 2011. The new version of the position statement, comparing to that of the formerly published one in 2009, implies some new data which are currently reviewed. Besides target value of antidiabetic treatment the importance of the target range is emphasized. Details about the monogenic forms of diabetes and the role of the continuous glucose monitoring system are discussed. A treatment algorithm for type 2 diabetes is published and the use of newly available antidiabetic drugs is summarized. Finally, the importance of the early diagnosis and the prevention of diabetes mellitus are pointed out.]

Lege Artis Medicinae

[Fulvestrant therapy in elderly: long survival, good quality of life]

UHLYARIK Andrea, RIEDL Erika, ÁGOSTON Péter, SARKADI Gábor, PÁPAI Zsuzsanna

Lege Artis Medicinae

[Homeostasis - The art of life and equilibrium]

KAPÓCS Gábor

All articles in the issue

Related contents

Lege Artis Medicinae

[Autoimmune pancreatitis in Hungary: a national multicenter study]

CZAKÓ László, GYÖKERES Tibor, TAKÁCS Tamás, TOPA Lajos, SAHIN Péter, DUBRAVCSIK Zsolt, SZEPES Attila, PAP Ákos, FÖLDESI Imre, TISZLAVICZ László, WITTMANN Tibor

[BACKGROUND - Autoimmune pancreatitis (AIP) is an increasingly recognised, special form of chronic pancreatitis, which greatly differs from other forms of chronic pancreatitis. Most papers on this condition have been published in Japan. METHODS - In our multicenter study, we aim to present the characteristics (demographics, clinical symptoms, laboratory and morphological findings, extrapancreatic symptoms, response to therapy, remission) of the first 13 Hungarian cases of AIP. RESULTS - The mean age at presentation was 44.2 years (range: 19-74); 54% of patients were women. New-onset mild abdominal pain (77%), weight loss (38%) and jaundice (31%) were the most common symptoms, with ulcerative colitis as the most frequent (38%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (54%), and a focal mass in 5 (38%). Pancreatic duct strictures were present in all patients. Serum immunoglobulin-G4 level was elevated in 71% of the patients in whom it was measured. All percutaneous core biopsies (4 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed hystological findings typical for AIP: periductal, diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. Immunostaining revealed IgG4-positive plasma cells in 57% of the patients in whom it was examined. Granulocytic epithelial lesions (GEL) were detected in 3 patients. These patients were younger (mean age 34 years), 66% were women and 6% had ulcerative colitis, whereas the mean age of patients without GEL was 65 years, and the majority of them were men. Steroid treatment resulted in remission of the symptoms in all patients. Because of suspicion of pancreatic tumour, 2 patients with focal AIP underwent partial pancreatectomy. Symptoms relapsed in one patient, but an increased dose of steroid resulted in a remission, which was maintained by azathioprine therapy. CONCLUSIONS - In our first Hungarian cases, we have confirmed previously reported characteristics of AIP. AIP with GEL is frequent among our patients; this condition is more frequent among women and younger patients, and is often associated with ulcerative colitis. If AIP is suspected, the performance of percutaneous biopsy is highly recommended. The therapeutic response to steroid therapy was excellent.]

Lege Artis Medicinae

[AUTOIMMUNE PANCREATITIS - AN UNDERDIAGNOSED DISEASE?]

CZAKÓ László

[Autoimmune pancreatitis is a recently recognized type of chronic pancreatitis that is clearly distinct from alcoholic chronic pancreatitis. Its clinical symptoms include jaundice, abdominal pain, weight loss and diabetes mellitus. It may be associated with other autoimmune diseases. IgG levels are elevated and autoantibodies can be detected. Pancreatic imaging reveals a diffuse enlargement of the pancreas and irregular narrowing of the main pancreatic duct. The characteristic histological features are lymphoplasmacytic infiltration and fibrosis. Autoimmune pancreatitis responds dramatically to steroid therapy, in contrast to other types of chronic pancreatitis, which hardly respond to any of the various therapies. It is important to be aware of this disease because it may be mistaken for other forms of chronic pancreatitis or pancreatic cancer, which leads to pancreatic resection when steroid treatment would be sufficient. This review discusses the clinical, laboratory, histological and imaging findings that are seen in autoimmune pancreatitis with particular focus on diagnosis. With the improvement of the diagnostic work-up less unnecessary pancreatic resections are expected to happen in patients with autoimmune pancreatitis.]

Clinical Neuroscience

Creutzfeldt-Jakob Disease: A single center experience and systemic analysis of cases in Turkey

USLU Ilgen Ferda, ELIF Gökçal, GÜRSOY Esra Azize, KOLUKISA Mehmet, YILDIZ Babacan Gulsen

We aimed to analyze the clinical, laboratory and neuroimaging findings in patients with sporadic Creutzfeldt-Jakob disease (CJD) in a single center as well as to review other published cases in Turkey. Between January 1st, 2014 and June 31st, 2017, all CJD cases were evaluated based on clinical findings, differential diagnosis, the previous misdiagnosis, electroencephalography (EEG), cerebrospinal fluid and cranial magnetic resonance imaging (MRI) findings in our center. All published cases in Turkey between 2005-2018 were also reviewed. In a total of 13 patients, progressive cognitive decline was the most common presenting symptom. Two patients had a diagnosis of Heidenhain variant, 1 patient had a diagnosis of Oppenheimer-Brownell variant. Seven patients (53.3%) had been misdiagnosed with depression, vascular dementia, normal pressure hydrocephalus or encephalitis. Eleven patients (87%) had typical MRI findings but only 5 of these were present at baseline. Asymmetrical high signal abnormalities on MRI were observed in 4 patients. Five patients (45.4%) had periodic spike wave complexes on EEG, all appeared during the follow-up. There were 74 published cases in Turkey bet­ween 2005 and 2018, with various clinical presentations. CJD has a variety of clinical features in our patient series as well as in cases reported in Turkey. Although progressive cognitive decline is the most common presenting symptom, unusual manifestations in early stages of the disease might cause misdiagnosis. Variant forms should be kept in mind in patients with isolated visual or cerebellar symptoms. MRI and EEG should be repeated during follow-up period if the clinical suspicion still exists.