IgG4-related disease coexisting with autoimmune haemolytic anaemia.
TL;DR: The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone and the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed.
Abstract: An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only. Nevertheless, the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed. The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone.
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TL;DR: It is suggested that malignancy is associated with the subsequent development of IgG4-RD in a subset of patients with IgG3-RD, a fibroinflammatory disease of unclear etiology.
Abstract: Background IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unclear etiology 1 . Some studies suggest that IgG4-RD predisposes patients to malignancy or is a forme fruste of cancer 2 , but we have frequently observed IgG4-RD patients with a history of malignancy preceding the clinical onset of IgG4-RD. Objectives We sought to determine whether a history of malignancy was more common among patients at the onset of IgG4-RD compared to controls. Methods We identified IgG4-RD patients with a history of invasive malignancy from a well-defined cohort of 125 patients and compared their malignancy history to those of two reference groups. First, we calculated a standardized prevalence ratio against general US population estimates from the Surveillance, Epidemiology, and End Results (SEER) database. Second, we identified up to five age- and gender-matched controls for each case and calculated the odds of malignancy among those with IgG4-RD compared to controls using conditional logistic regression. Results The mean age at IgG4-RD onset was 50.3±14.9 years and 61% of the patients were male. Twenty (16%) had been diagnosed with 21 malignancies before the diagnosis of IgG4-RD. The observed prevalence of malignancy in this cohort was 2.5 times higher (95% CI:1.1–3.6) than expected compared to the SEER database. Compared with matched controls, a history of malignancy was more than three-fold higher in IgG4-RD (OR 3.1;95% CI:1.6–6.2). Conclusions Our findings suggest that malignancy is associated with the subsequent development of IgG4-RD in a subset of patients with IgG4-RD. Potential explanations include shared risk factors for both IgG4-RD and cancer, the triggering by cancer of autoantigen expression leading to IgG4-RD, and an increased risk of IgG4-RD resulting from cancer treatment. References Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–551. Hart PA, Law RJ, Dierkhising RA, Smyrk TC, Takahashi N, Chari ST. Risk of cancer in autoimmune pancreatitis: A case-control study and review of the literature. Pancreas. 2014;43(3):417–421. Disclosure of Interest None declared
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TL;DR: A systematic review of the literature of this new and challenging entity of cutaneous IgG4-RD, which presents as papules, plaques, and nodules involving the head and neck areas, is provided.
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TL;DR: In this paper, a case of a patient with IgG4-RD who had involvement of multiple organs: the kidneys, lymph nodes, bone marrow (biopsy performed), lungs, liver, and small intestine (imaging abnormalities).
Abstract: IgG4-related disease (IgG4-RD) is an immune-mediated multi-organ inflammatory disorder caused by tissue infiltration of lymphocytes with IgG4-secreting plasma cells. Herein, we discuss a case of a patient with IgG4-RD who had involvement of multiple organs: the kidneys, lymph nodes, bone marrow (biopsy performed), lungs, liver, and small intestine (imaging abnormalities). Although several case reports and series of IgG4-RD involving different organ involvement are in the literature, our patient has extensive simultaneous multi-organ involvement. We utilized the four domains (serologic, pathologic, radiologic, and pathologic) as discussed in the new 2019 ACR/EULAR classification criteria to provide a useful framework in considering an alternative tool for IgG4-RD in multi-organ involvement, where biopsy is more invasive and not always accessible. We highlight the findings of each organ involved that increase the likelihood that the patient has IgG4-RD. In our patient, the IgG4-RD classification criteria was fulfilled with total points adding up to 48. Our case meets the classification criteria for IgG4-RD, since at least one organ is involved to meet entry criteria (biopsy proven), no exclusion criteria are present, and the total points are ≥ 20. When such extensive involvement of IgG4-RD occurs, early diagnosis and treatment are recommended to avoid irreversible organ damage and better outcomes.
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TL;DR: IgG4 asociovana onemocněni představuji relativný novou a poměrný vzacnou skupinu systemových zanětlivý ch onemociým as discussed by the authors.
Abstract: IgG4 asociovana onemocněni představuji relativně novou a poměrně vzacnou skupinu systemových zanětlivých onemocněni, ktera je charakterizovana zanětlivým, fibrotizujicim ci sklerotizujicim postiženim jednoho nebo vice organů doprovazene lymfoplazmocelularni infiltraci tkani s výrazným zastoupenim IgG4 plazmatických buněk a větsinou zvýsenou koncentraci serových IgG4 imunoglobulinů (dle definice > 1,35 g/l; referencni rozmezi 0,08-1,40 g/l). Pro stanoveni diagnozy je klicový histopatologický nalez. Autoři prezentuji kazuistiku pacienta s IgG4 asociovaným systemovým onemocněnim projevujicim se vzacnou kombinaci autoimunitni hemolyticke anemie a plicniho postiženi napodobujiciho radiologicky metastatický proces.
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TL;DR: A 72-year-old woman referred to diabetes services with rapidly increasing blood glucose and weight loss despite oral hypoglycaemic therapy is reported, highlighting the importance of a detailed diagnostic work-up and describing an unusual clinical presentation of this increasingly recognised multisystem disease.
Abstract: Hyperglycaemia is one of the most common metabolic disturbances encountered in clinical practice, with an important differential diagnosis. We report a case of a 72-year-old woman referred to diabetes services with rapidly increasing blood glucose and weight loss despite oral hypoglycaemic therapy. She reported mild upper abdominal discomfort and liver function tests were deranged, prompting further investigation. Abdominal imaging demonstrated a diffusely enlarged pancreas and subsequent investigations noted a markedly raised serum IgG4. She was diagnosed with IgG4-related pancreatitis and swiftly responded to steroid therapy. Secondary causes of diabetes should be considered in people with atypical presentation such as weight loss or rapid progression to insulin use. While IgG4-related disease is rare, its varied clinical presentation as a result of its multiorgan involvement requires a high index of suspicion. This case highlights the importance of a detailed diagnostic work-up and describes an unusual clinical presentation of this increasingly recognised multisystem disease.
1 citations
References
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TL;DR: The categorization of AIP into types 1 and 2 should be helpful for further clarification of the clinical features, pathogenesis, and natural history of these diseases.
Abstract: Objectives:To achieve the goal of developing international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP).Methods:An international panel of experts met during the 14th Congress of the International Association of Pancreatology held in Fukuoka, Japan, from July 11 through 13,
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TL;DR: Advances in understanding the pathophysiology of IgG4-RD have proceeded swiftly, leading to the identification of a number of potential targeted treatment strategies, which have direct implications for the development of targeted strategies for the treatment of this condition.
Abstract: Purpose of review Remarkable insights have been gleaned recently with regard to the pathophysiology of IgG4-related disease (IgG4-RD). These findings have direct implications for the development of targeted strategies for the treatment of this condition. Recent findings Oligoclonal expansions of cells of both the B and T lymphocyte lineages are present in the blood of patients with IgG4-RD. Oligoclonal expansions of plasmablasts are a good biomarker for disease activity. An oligoclonally expanded population of CD4+ cytotoxic T lymphocytes is found not only in the peripheral blood but also at tissue sites of active disease. This cell elaborates cytokines that may drive the fibrosis characteristic of IgG4-RD. T follicular helper cells (Tfhc), particularly the Tfhc2 subset, appear to play a major role in driving the class switch to IgG4 that typifies this disease. The relationship between malignancy and IgG4-RD remains an area of interest. Summary Advances in understanding the pathophysiology of IgG4-RD have proceeded swiftly, leading to the identification of a number of potential targeted treatment strategies. The completion of classification criteria for IgG4-RD, an effort supported jointly by the American College of Rheumatology and the European League Against Rheumatism, will further facilitate studies on this disease.
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TL;DR: Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.
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TL;DR: To the Editor: autoimmune pancreatitis is a recently recognized disorder and a new clinical entity associated with the irregular narrowing of the pancreatic duct and the enlargement of the entire pancreas.
Abstract: To the Editor:Autoimmune pancreatitis (AIP) is a recently recognized disorder and a new clinical entity associated with the irregular narrowing of the pancreatic duct and the enlargement of the entire pancreas.1 AIP is often associated with sclerosing cholangitis (SC). SC with AIP and primary sclero
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01 Jul 2015
TL;DR: Most recent knowledge about the clinical, laboratory, radiological, and pathological characteristics of IgG4-RD that may guide the physician to establish an early diagnosis are provided.
Abstract: IgG4-related disease (IgG4-RD) is a systemic disease characterized by the infiltration of IgG4-bearing plasma cells and, more importantly, distinctive histopathological features: storiform fibrosis, obliterative phlebitis, a lymphoplasmacytic infiltrate, and mild-to-moderate tissue eosinophilia. The diagnostic approach is complex and relies on the coexistence of various clinical, laboratory, and histopathological findings, none of which is pathognomonic in and of itself. IgG4-related disease should be suspected in patients presenting with unexplained enlargement or swelling of 1 or more organs or tissue organs. Four laboratory abnormalities often provide initial clues to the diagnosis of IgG4-RD: peripheral eosinophilia, hypergammaglobulinemia, elevated serum IgE levels, and hypocomplementemia. Elevated serum IgG4 levels provided critical information in identifying the first cases of IgG4-RD, but recent studies have reported substantial limitations to the measurement of serum IgG4 concentrations, precluding reliance on serum IgG4 concentrations for diagnostic purposes. In contrast, new studies have suggested a promising role of flow cytometry studies in the diagnosis and longitudinal management of IgG4-RD. Demonstration of the classic histopathological features of IgG4-RD remains crucial to diagnosis in most cases, and biopsy proof is preferred strongly by most disease experts before the initiation of treatment. Of note, the multiorgan nature of IgG4-RD was first established in 2003. This review intends to provide most recent knowledge about the clinical, laboratory, radiological, and pathological characteristics of IgG4-RD that may guide the physician to establish an early diagnosis. We searched PubMed and MEDLINE for relevant articles published between January 1, 2000, and November 1, 2014, using the search terms IgG4 and IgG4-related.
149 citations