The variety of clinical presentations in IgG4-related disease in Rheumatology

Sebastian, Agata; Sebastian, Maciej; Misterska-Skóra, Maria; Donizy, Piotr; Hałoń, Agnieszka; Chlebicki, Arkadiusz; Lipiński, Artur; Wiland, Piotr

doi:10.1007/s00296-017-3807-1

Cited by 11 publications

(6 citation statements)

References 30 publications

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“…Indeed, IgG4-RD may potentially affect any organ/tissue, such as lacrimal and salivary glands, lymph nodes, orbital or periorbital tissue, breast, skin, pancreas, biliary tract, thyroid, mediastinum, lungs, pleura, pericardium, aorta, arteries, retroperitoneum, kidneys, prostate, pachymeninx, hypophysis. [ 16 , 17 ]…”

Section: Discussionmentioning

confidence: 99%

Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease

et al. 2018

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Rationale:IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease characterized by multi-organ involvement and variable clinical behavior.Patient concerns:We describe the case of a 50-year-old woman affected by a rare variant of IgG4-RD, characterized by eyelid xanthelasmas, adult-onset asthma and salivary and lacrimal glands enlargement. Multiple lymphadenopathies and a pulmonary mass were present at initial evaluation.Inteventions:After a single course of rituximab (2g in 2 refracted doses), an almost complete clinical remission was achieved without chronic steroid administration.Outcomes:Magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) of the thorax, and positron emission tomography (18FDG-PET-CT) confirmed good response to treatment. Circulating plasmablasts dropped to undetectable levels as well. Xanthelasmas only remained unchanged. Remission persisted at 1-year follow-up.Lessons:Steroid therapy is still considered standard first-line therapy in IgG4-RD. However, high doses are generally required and relapses are common during the tapering phase. Rituximab is a well described steroid-sparing strategy, so far reserved to refractory cases only. In our experience, rituximab has been used as first-line monotherapy, showing great and sustained efficacy and optimal tolerability. The peculiar variant of IgG4-RD affecting our patient, the relatively low baseline plasmablast concentration, and the early placement of rituximab therapy may have facilitated the good response.

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Section: Discussionmentioning

confidence: 99%

Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease

et al. 2018

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“…IgG4-залежне захворювання (IgG4-33) виділено в окрему нозологічну одиницю близько 10 років. У 2012 році було прийнято уніфіковану номенклатуру IgG4-33, запропоновано діагностичні критерії [17,18]. IgG4-33 уражає осіб середнього та старшого віку (50-70 років), хоча є і у дітей.…”

Section: медичні наукиunclassified

“…Рівень IgG4-33 підвищений при синдромі Шегрена, ситемному червоному вовчаку, ревматоїдному артриті (у 46%), причому підвищений рівень часто корелює з активністю захворювання. Підвищення рівня IgG4-33 зустрічається також при раку ПЗ, еозинофільному гранульоматозі з поліангіїтом, саркоїдозі, а також у здоровій популяції [18].…”

Section: медичні наукиunclassified

Pecularities of the Pancreatitis Development on the Background of Some Rheumatic Diseases

Teleki¹,

Khrystych²,

Olinik³

et al. 2022

Ukr. ž. med. bìol. sportu

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The purpose of the study was to evaluate the main pathogenetic mechanisms of pancreatic lesions on the background of systemic connective tissue diseases based on the analytical analysis of modern literature data. Materials and methods. Bibliosemantic and analytical methods were used in the research. Results and discussion. The main pathogenetic link of the pancreatitis development in patients with systemic connective tissue diseases is vasculitis of the pancreas. Increased expression of adhesion molecules with activation of leukocytes and endothelial cells, deposition of circulating immune complexes in the vascular wall, production of antibodies to endothelial cells, capillary basement membranes play an important role in this process. In systemic lupus erythematosus, according to various authors, the frequency of arteritis varies greatly: rates range from 6.2-7.4 to 53%. In rheumatoid arthritis, the frequency of arteritis of the pancreatic vessels reaches 50%, in systemic sclerosis – 17%. Secondary Sjogren's syndrome is associated with autoimmune pancreatitis in a quarter of cases, but is not the cause. In diseases such as rheumatoid arthritis, systemic scleroderma and systemic lupus erythematosus, antibodies that can attack phospholipids of cell membranes are produced. Antiphospholipid syndrome develops often in systemic lupus erythematosus (70% of cases). In rheumatic fever patients’ changes in the pancreas were studied only in single studies. The main mechanism of pathogenesis of both acute and chronic pancreatitis in nodular periarteritis is the involvement of small and medium arteries of the pancreas in the pathological process. In granulomatous polyangiitis in the pancreas reveals vascular-granulomatous changes, resulting in the formation of extravasations, necrotic foci, foci of atrophy, sclerosis. In IgA vasculitis, changes in the structure of the pancreas are minimal or there are isolated small subcapsular hemorrhages. It is established that metabolic disorders occur in many rheumatic diseases. Thus, reduced glucose tolerance is observed in 7–74% of patients, hypercholesterolemia and triglyceridemia – in 50–75%, hypertension – in 25–50% of cases. Conclusion. Thus, the diagnostic approach to the pancreatitis in systemic connective tissue diseases is very difficult. Its manifestations are masked by damage of the other organs. The availability of more sensitive diagnostic methods, their accessibility can provide an opportunity to detect symptoms of pancreatitis earlier, which will contribute to the appointment of optimal treatment, improvement of the prognosis, quality of life and survival of such patients

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“…Початок IgG4-ЗЗ зазвичай є підгострим. У хворих часто відсутня будь-яка симптоматика, і захворювання діагностується випадково під час обстеження з приводу інших причин [7]. Можлива також спонтанна ремісія з багаторічною відсутністю активності захворювання [8].…”

Section: патоморфологія класифікація та клінічні прояви Igg4-ззunclassified

IgG4-related kidney disease

Iaremenko

Koliadenko

2021

KIDNEYS

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IgG4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease characterized by the formation of sclerotic tumor-like masses with dense lymphoplasmacytic infiltrates containing a significant number of IgG4-plasma cells. IgG4-RD is characterized by a wide range of clinical manifestations since the disease can involve almost any organ. Renal damage is observed in about 15 % of patients with IgG4-RD. The article presents a review of data about clinical manifestations of IgG4-related kidney disease (IgG4-RKD), specific features of diagnosis, and modern treatment approaches. The review of scientific publications was conducted in the international electronic scientometric database PubMed using keywords “IgG4-related kidney disease”, “IgG4-RKD radiographic findings”, “IgG4-related tubulointerstitial nephritis”, “IgG4-related membranous glomerulonephritis” over the period 2012–2020. The most common renal lesions in IgG4-RD are tubulointerstitial nephritis and membranous glomerulonephritis. They occur predominantly in older males. At the time of diagnosis, patients mostly already have concomitant extrarenal manifestations of IgG4-RD (sialadenitis, pancreatitis, lymphadenopathy). The clinical picture is usually vague, although nephrotic syndrome and chronic renal failure may occur. Typical manifestations of IgG4-RKD on CT are multiple low-dense foci, thickening of the renal pelvis, diffuse kidney enlargement. Renal involvement in IgG4-RD is often accompanied by low levels of complement. Early detection and treatment of IgG4-RKD are extremely important due to the increase in morbidity and mortality due to the development of chronic kidney disease. Glucocorticoids (GC) are first-line drugs for IgG4-RD. Despite a good response to GC, even a slight delay in treatment can lead to the development of renal tissue atrophy. The use of immunosuppressive drugs is advisable in cases when the dose of GC cannot be reduced due to the constantly high activity of the disease. Rituximab (a CD20+ B-lymphocyte inhibitor) may be used in patients with a recurrent course of IgG4-RKD.

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The variety of clinical presentations in IgG4-related disease in Rheumatology

Cited by 11 publications

References 30 publications

Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease

Sustained clinical response after single course of rituximab as first-line monotherapy in adult-onset asthma and periocular xanthogranulomas syndrome associated with IgG4-related disease

Pecularities of the Pancreatitis Development on the Background of Some Rheumatic Diseases

IgG4-related kidney disease

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