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Lege Artis Medicinae

DECEMBER 10, 2018

[IgG4-related gastropathy mimicking malignancy]

NAGY Pál, KASZÁS Ilona, PÁK Gábor, TIBA Imre, FELFÖLDI Éva, HAMVAS József, BAGDI Enikő, KRENÁCS László

[The Immunoglobulin G4-related disease (IgG4-RD) along with its synonyms is recently a popular topic in the medical literature. This illness can affect almost every organ in the body therefore it is frequently discussed in any type of interdisciplinary forums. We presented an instructive case of IgG4-RD with gastric involvement in 2016. Hereby we share our pertinent experiences. We are not dealing with the IgG4-RD in general including its terminology problems, epidemiology, pathogenesis, detailed clinical/pathological appearances, diagnostic criteria and treatment. A 63-year-old male presented with symp­toms of pylorus stenosis. The endoscopic findings and CT images were interpreted as those of a malignant tumour in the antrum of the stomach. So a subtotal resection was performed although the biopsy showed reactive proliferation of plasma cells without evidence of malignancy. Pathological examination of the surgical specimen confirmed an IgG4- associated sclerosing inflammation. The patient had an uneventful recovery. Our case is an example of rare, isolated formof IgG4-RD. ]

Hypertension and nephrology

DECEMBER 10, 2013

[Current diagnosis and treatment of membranous nephropathy]

STUDINGER Péter, CSEPREKÁL Orsolya, FINTHA Attila, KARDOS Magdolna, TISLÉR András

[Primary membranous nephropathy is a common glomerular disorder characterized by subepithelial immune deposits. The pathomechanism underlying these lesions has only recently been elucidated: M-type phospholipase A2receptor (PLA2R) protein emerged as being the leading autoantigen. Antibodies to PLA2R, typically of IgG4 subclass are expressed in 70-80% of patients with primary membranous nephropathy. The level of autoantibody to PLA2R was shown to correlate with disease severity and to change parallel with disease activity in response to therapy. While mild forms of the disease are prone to spontaneous remission and carry excellent prognosis, severe forms often progress into end-stage renal disease without treatment and necessitate immunosuppression. The latest guidelines recommend the application of corticosteroids with alkylating agents or calcineurin inhibitors as first-line therapy. Promising new therapies that are currently being explored for this disease include rituximab, mycophenolate mofetil, and adrenocorticotropic hormone.]

Lege Artis Medicinae

JUNE 21, 2006

[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.]

Lege Artis Medicinae

JANUARY 20, 2011

[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

APRIL 22, 2011

[IgG4-related disease]

ZEHER Margit

[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.]