Tissue Inhibitors of Metalloproteinase 1 (TIMP-1) and 3 (TIMP-3) as New Markers of Acute Kidney Injury After Massive Burns.

BACKGROUND Acute kidney injury (AKI) is a common and serious complication after massive burn injury. One of the postulated etiologies is destruction of the extracellular matrix of nephrons, caused by a local imbalance between matrix metalloproteinases (MMPs) and specific inhibitors. The aim of this study was to analyze the dynamics of tissue inhibitors of metalloproteinases (TIMPs) during the first 5 days after massive thermal injury and the relationship with the risk of AKI. MATERIAL AND METHODS Thirty-three adults (22 men, 11 women) with severe burns were enrolled in the study. The values of TIMPs 1 to 4 were measured in blood serum and urine using the multiplex Luminex system. The associations between TIMPs and the risk of AKI were analyzed by using the generalized linear mixed models for repeated measurements. RESULTS Significant changes in serum and urine activities of TIMPs were confirmed, especially during the first 2 days after burn injury. Almost half of patients presented renal problems during the study. Significant differences between values of TIMPs in AKI and non-AKI status were also observed. However, a significant relationship between concentration of TIMPs and risk of AKI was confirmed only for urine TIMP-1 and serum TIMP-3. CONCLUSIONS The evaluation of TIMPs in the early stage after burn injury has potential benefits. The important roles of urine TIMP-1 and serum TIMP-3, as novel markers of the risk of AKI development, were confirmed. Other parameters require further analysis.

Introduction to Amniotic Membranes in Maxillofacial Surgery-A Scoping Review.

Background: Amniotic membrane (AM) holds significant promise in various medical fields due to its unique properties and minimal ethical concerns. This study aims to explore the diverse applications of the human amniotic membrane (HAM) in maxillofacial surgery. Methodology: A comprehensive search was conducted on databases, namely Google Scholar, PubMed, and Scopus, from January 1985 to March 2024. Articles in English, Polish, and Spanish were included, focusing on keywords related to amniotic membrane and oral surgery. Results: Various preservation methods for HAM were identified, namely fresh, decellularized, cryopreserved, lyophilized, and air-dried formats. Clinical studies demonstrated the efficacy of HAM in repairing oral mucosal defects, vestibuloplasty, oronasal fistula closure, cleft palate treatment, bone defect repair, and medication-related osteonecrosis of the jaw (MRONJ). Surgeon evaluations highlighted the ease of handling but noted challenges in suturing and stability during application. Conclusions: Amniotic membranes offer a versatile and effective option in maxillofacial surgery, promoting wound healing, reducing inflammation, and providing a scaffold for tissue regeneration. Further research, including randomized trials and comparative studies, is warranted to validate the efficacy and optimize the utilization of HAM in clinical practice.

Platelet-Rich Plasma - a remedy present in every human being. History, functioning, and the benefits of therapy using it.

Platelet-rich plasma is an autologous product used in restorative medicine. It contains a high concentration of platelets, which are rich in growth factors and other biologically active substances known for their ability to stimulate regenerative processes in the body. Currently, research is being conducted into the use of platelet-rich plasma in many areas of medicine. This publication provides information on the nature, mechanism of action, therapeutic properties and application of autologous platelet-rich plasma in medicine. Furthermore, ongoing investigations explore its potential in wound healing, orthopedics, dermatology, and even in dentistry, showcasing its versatility and promising outcomes across various medical disciplines. Additionally, the safety and efficacy of platelet-rich plasma therapies are subjects of continual scrutiny, aiming to refine protocols and expand its clinical utility with robust scientific evidence. The growing interest in this regenerative approach underscores its potential as a valuable tool in modern medical practice. Platelet-rich plasma therapy represents a promising avenue for personalized medicine, offering tailored treatment approaches that capitalize on the body's own healing mechanisms to promote tissue repair and regeneration.

Different Types of Vasculitis Complicated by Heparin-Induced Thrombocytopenia-Analysis of Four Cases and Literature Review.

Vasculitis and HIT have different etiologies, although both involve autoimmune mechanisms. Treatment of vasculitis often requires the use of an anticoagulant such as heparin, which can lead to the development of HIT and subsequent life-threatening complications. The analysis covered patients hospitalized in the Department of Internal Medicine, Nephrology and Dialysis in the period from September 2020 to March 2023. After analyzing the data, we selected four patients in whom vasculitis treatment was complicated by HIT. These included two patients with ANCA vasculitis and two patients with anti-GBM disease. We also described similar cases reported in the literature.

IMPACT OF E-CIGARETTES ON THE ORAL HEALTH - LITERATURE REVIEW.

E-cigarettes are electronic devices used to inhale aerosols generated from the vaporization of flavored liquids. Nowadays, the use of e-cigarettes has become one of the most popular alternatives to traditional smoking. The wide variety of devices and liquids makes it challenging to assess the health effects of using e-cigarettes. During the vaporization of e-liquids, toxins, carcinogens, and various other chemicals can be released and inhaled by the user. Limited data exist regarding the potential health impact of exposure to e-vapors, primarily derived from animal studies and in vitro research. The oral tissues are the first site of direct interaction with the components of the inhaled vapor. While e-cigarettes are commonly portrayed as safer alternatives to tobacco cigarettes, little is known about the short- or long-term health effects of their use. The aim of this review is to briefly present the available data regarding the impact of chemical ingredients and toxins present in e-cigarette vapors on oral cavity cells.

Ocular Involvement of Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's disease, is a form of ANCA-associated vasculitis. It manifests mainly in the kidneys and the upper respiratory tract, but ocular involvement is not uncommon. In this article, four cases with ocular manifestations are presented with comprehensive photographic documentation. We describe the way to proper diagnosis, which may be long, the possible treatment, and the final outcomes. Our patients had the following ocular manifestations of GPA: retinal vasculitis, anterior necrotizing scleritis, medial orbital wall and orbital floor erosion with middle face deformation, compressive optic neuropathy due to retrobulbar inflammatory mass, and the abscess of the eyelids, inflammatory intraorbital mass causing exophthalmos and diplopia. This manuscript includes the description of severe forms of GPA, the initial signs and symptoms, relapses, and difficulties in achieving remission. The extraocular involvement is described with diagnostic modalities and laboratory findings. One of the reported cases was diagnosed by an ophthalmologist on the basis of ocular symptoms in the early stages of the disease. Our outcomes are compared with those discussed in the literature.

The Review of Current Knowledge on Neutrophil Gelatinase-Associated Lipocalin (NGAL).

Neutrophil gelatinase-associated lipocalin (NGAL) is a 25-kDa protein that is secreted mostly by immune cells such as neutrophils, macrophages, and dendritic cells. Its production is stimulated in response to inflammation. The concentrations of NGAL can be measured in plasma, urine, and biological fluids such as peritoneal effluent. NGAL is known mainly as a biomarker of acute kidney injury and is released after tubular damage and during renal regeneration processes. NGAL is also elevated in chronic kidney disease and dialysis patients. It may play a role as a predictor of the progression of renal function decreases with complications and mortality due to kidney failure. NGAL is also useful in the diagnostic processes of cardiovascular diseases. It is highly expressed in injured heart tissue and atherosclerostic plaque; its serum concentrations correlate with the severity of heart failure and coronary artery disease. NGAL increases inflammatory states and its levels rise in arterial hypertension, obesity, diabetes, and metabolic complications such as insulin resistance, and is also involved in carcinogenesis. In this review, we present the current knowledge on NGAL and its involvement in different pathologies, especially its role in renal and cardiovascular diseases.

Heart Metastases of Clear Cell Renal Cell Carcinoma.

Renal cell carcinoma (RCC) is a common genitourinary cancer. Of the several histologic types of RCC, clear cell renal cell carcinoma (ccRCC) is the most frequent. Due to the development of imaging methods such as computed tomography (CT) or magnetic resonance imaging (MRI), the incidence of ccRCC diagnosis has increased rapidly. However, up to one third of patients at prime diagnosis of ccRCC are at metastatic stadium of the disease. Metastases of ccRCC are found mostly in the lungs, bones and liver. Metastasis of ccRCC to the heart is an uncommon clinical situation. We present a rare case of metastatic stadium of ccRCC with metastases to heart tissue visualized in transthoracic echocardiography.

Resistin Contribution to Cardiovascular Risk in Chronic Kidney Disease Male Patients.

BACKGROUND: Resistin is a molecule that belongs to the Resistin-Like Molecules family (RELMs), the group of proteins taking part in inflammatory processes. Increased resistin concentrations are observed in cardiovascular complications. Resistin contributes to the onset of atherosclerosis and intensifies the atherosclerotic processes. The aim of this study was to investigate the relationship between resistin and cardiovascular (CV) risk in men with chronic kidney disease (CKD) not treated with dialysis. MATERIALS AND METHODS: One hundred and forty-two men were included in the study: 99 men with eGFR lower than 60 mL/min/1.73 m2 and 43 men with eGFR ≥ 60 mL/min/1.73 m2. CV risk was assessed. Serum resistin, tumor necrosis factor-alpha (TNF-alpha) and plasminogen activator inhibitor-1 (PAI-1) were measured among other biochemical parameters. RESULTS: We observed that resistin concentrations were significantly higher in patients with CKD compared to individuals with eGFR ≥ 60 mL/min/1.73 m2 (p = 0.003). In CKD, after estimating the general linear model (GLM), we found that resistin is associated with CV risk (p = 0.026) and PAI-1 serum concentrations (0.012). The relationship of PAI-1 with resistin depends on the level of CV risk in CKD (p = 0.048). CONCLUSIONS: Resistin concentrations rise with the increase of CV risk in CKD patients and thus resistin may contribute to the progression of cardiovascular risk in this group of patients. The relationship between resistin and CV risk is modified by PAI-1 concentrations.

Inferior Nutritional Status Significantly Differentiates Dialysis Patients with Type 1 and Type 2 Diabetes.

Diabetes mellitus is currently the leading cause of end-stage renal disease. Assessing nutritional status is an important component of care in this group. This prospective observational study aimed to assess the nutritional status of type 1 and type 2 diabetes patients on hemodialysis or peritoneal dialysis and its relationship with hospitalizations and all-cause death. Adult patients with end-stage renal disease, treated with dialysis, and suffering from type 1 or type 2 diabetes, being treated with insulin, were included in the study. Exclusion criteria comprised other types of diabetes, the patient's refusal to participate in the study, and severe disorders impacting verbal-logical communication. The nutritional status based on the Nutritional Risk Index, the Geriatric Nutritional Risk Index, fat distribution measures, and the Charlson Comorbidity Index was estimated for 95 Caucasian dialysis patients with type 1 (n = 25) or type 2 (n = 70) diabetes. Patients with type 1 diabetes exhibited significantly inferior nutritional status and increased nutritional risk than those with type 2 diabetes. Lower values of nutritional indices significantly differentiated patients with type 1 from those with type 2 diabetes, with ≥84% sensitivity and specificity. Inferior nutritional status was related to all-cause hospitalizations, whereas higher comorbidity was associated with a greater likelihood of cardiovascular hospitalizations and all-cause death. The significant difference between patients with type 1 and type 2 diabetes being treated with dialysis indicates that these patients should not be considered as a homogeneous group, while also considering the greater age of patients with type 2 diabetes.

Ultrasound Doppler Flow Parameters Are Independently Associated with Renal Cortex Contrast-Enhanced Multidetector Computed Tomography Perfusion and Kidney Function.

BACKGROUND: The assessment of kidney perfusion has an emerging significance in many diagnostic applications. However, whether and which of the ultrasound Doppler parameters better express renal cortical perfusion (RCP) was not shown. The study aimed to prove the usefulness of Doppler ultrasound parameters in the assessment of RCP regarding low-dose contrast-enhanced multidetector computer tomography (CE-MDCT) blood flow. METHODS: Thirty non-stenotic kidneys in twenty-five hypertensive patients (age 58.9 ± 19.0) with mild-to-severe renal dysfunction were included in the study. Resistive index (RI) and end-diastolic velocity (EDV) in segmental arteries, color Doppler dynamic RCP intensity (dRCP), RI (dRI), pulsatility index (dPI), and CE-MDCT blood flow (CBF) in the renal cortex were estimated. RESULTS: CBF correlated significantly with age, estimated glomerular filtration rate (eGFR), RI, EDV, dRI, dPI, and dRCP. In separate multivariable backward regression analyses, RI (R2 = 0.290, p = 0.003) and dRCP (R2 = 0.320, p = 0.001) were independently associated with CBF. However, in the common ultrasound model, only dRCP was independently related to CBF (R2 = 0.317, p = 0.001). Only CBF and EDV were independently associated with eGFR (R2 = 0.510, p < 0.001). CONCLUSIONS: Renal cortical perfusion intensity is the best ultrasound marker expressing renal cortical perfusion. In patients with hypertension and kidney dysfunction, renal resistive index and end-diastolic velocity express renal cortical perfusion and kidney function, respectively.

Melatonin Treatment in Kidney Diseases.

Melatonin is a neurohormone that is mainly secreted by the pineal gland. It coordinates the work of the superior biological clock and consequently affects many processes in the human body. Disorders of the waking and sleeping period result in nervous system imbalance and generate metabolic and endocrine derangements. The purpose of this review is to provide information regarding the potential benefits of melatonin use, particularly in kidney diseases. The impact on the cardiovascular system, diabetes, and homeostasis causes melatonin to be indirectly connected to kidney function and quality of life in people with chronic kidney disease. Moreover, there are numerous reports showing that melatonin plays a role as an antioxidant, free radical scavenger, and cytoprotective agent. This means that the supplementation of melatonin can be helpful in almost every type of kidney injury because inflammation, apoptosis, and oxidative stress occur, regardless of the mechanism. The administration of melatonin has a renoprotective effect and inhibits the progression of complications connected to renal failure. It is very important that exogenous melatonin supplementation is well tolerated and that the number of side effects caused by this type of treatment is low.

Comparable Hemodilution with Hypertonic Glucose in Patients with and without Type-2 Diabetes Mellitus during Hemodialysis.

(1) Background: It was examined whether glucose-induced changes in the relative blood volume are suitable to identify subjects with and without type-2 diabetes mellitus (T2D) during hemodialysis. (2) Methods: The relative blood volume was continuously recorded during hemodialysis and perturbed by the infusion of glucose comparable to the dose used for intravenous glucose tolerance tests. Indices of glucose metabolism were determined by the homeostatic model assessment (HOMA). Body composition was measured by a bioimpedance analysis. The magnitude and the time course of hemodilution were described by a modified gamma variate model and five model parameters. (3) Results: A total of 34 subjects were studied, 14 with and 20 without T2D. The magnitude of the hemodilution and the selected model parameters correlated with measures of anthropometry, body mass index, absolute and relative fat mass, volume excess, baseline insulin concentration, and HOMA indices such as insulin resistance and glucose disposition in a continuous analysis, but were not different in a dichotomous analysis of patients with and without T2D. (4) Conclusions: Even though the parameters of the hemodilution curve were correlated with measures of impaired glucose metabolism and body composition, the distinction between subjects with and without T2D was not possible using glucose-induced changes in the relative blood volume during hemodialysis.

Low Free Triiodothyronine as a More Sensitive Predictor of Survival Than Total Testosterone among Dialysis Men.

BACKGROUND: Some endocrine disorders, previously considered benign, may be related to a poorer prognosis for patients with renal failure. Both low serum free triiodothyronine (fT3) and low total testosterone (TT) concentrations have been considered as predictors of death in dialysis patients, but the results of studies are inconsistent. In our study, we evaluated the relationships of the serum thyroid hormone levels and the total testosterone levels with survival in male dialysis patients. METHODS: Forty-eight male dialysis patients, 31 on hemodialysis (HD) and 17 on peritoneal dialysis (PD), aged 61.4 ± 10.0, 59.2 ± 12.2 years, respectively, were included in the study. Serum thyroid hormones and total testosterone were measured. RESULTS: During the 12-month follow-up, nine all-cause deaths were recorded. The concentrations of fT3 were significantly lower in those who died than in the survivors (p = 0.001). We did not observe any statistically considerable differences between the group of men who died and the rest of the participants in terms of the total serum testosterone concentration (p = 0.350). Total testosterone positively correlated with fT3 (r = 0.463, p = 0.009) in the HD group. CONCLUSIONS: In the group of male dialysis patients, the serum concentration of fT3 had a better prognostic value in terms of survival than the total testosterone. A linear relationship between the fT3 levels and testosterone levels in men undergoing hemodialysis may confirm the hypothesis that some of the hormonal changes observed in chronic kidney disease (CKD) may have a common cause.

Changes of Hemodynamic Parameters after Intradialytic Glucose Injection.

BACKGROUND: Intradialytic hypotension (IDH) is a frequent complication of hemodialysis (HD). Current methods of IDH prevention are insufficient. METHODS: We analyzed the intradialytic time course of systolic (SBP), diastolic (DBP), mean arterial (MAP), pulse pressure (PP), and heart rate (HR) in a group of chronic kidney disease (CKD) patients. First, 30 min into HD, a 40% glucose solution was injected into the venous line of the extracorporeal circulation at a dose of 0.5 g/kg of dry weight. Pressures and HR were measured in frequent intervals. Relative volume overload was determined by bioimpedance spectroscopy. RESULTS: Thirty-five participants were studied. SBP increased after 5, 10, and 20 min of glucose infusion. DBP increased after 2 and 3 h and also at the end of HD. PP increased after 5, 10, and 20 min of glucose infusion and fell after the 2nd and 3rd hour and also at the end of HD. MAP increased after 2 and 3 h of glucose injection and at the end of HD. Significant interactions of the time course of SBP, DBP, MAP, with HR at baseline and of the time course of PP with fluid overload were observed. Symptomatic hypotensive episodes were absent. CONCLUSIONS: Glucose infusions during HD prevent symptomatic IDH and do not cause severe hypertensive episodes.

Cardiovascular Response to Intravenous Glucose Injection during Hemodialysis with Assessment of Entropy Alterations.

BACKGROUND: The quality of autonomic blood pressure (BP) control can be assessed by the entropy of serial BP data. The aim of this study was to evaluate the effect of hemodialysis (HD) and glucose infusion (GI) on amplitude aware permutation entropy (AAPE) of hemodynamic variables during HD in chronic kidney disease patients with and without type-2 diabetes mellitus (DM). METHODS: Twenty-one patients without DM (NDO) and ten with DM were studied. Thirty minutes after the start of HD, a 40% glucose solution was administered. Hemodynamic data were extracted from continuous recordings using the Portapres® system. RESULTS: AAPE decreased during HD in all patients and all hemodynamic signals with the exception of AAPE of mean and diastolic BP in DM patients. GI led to an increase in AAPE for cardiac output in all patients, while AAPE for heart rate and ejection time increased only in DM studies, and AAPE for systolic, diastolic, and mean arterial pressure, as well as total peripheral resistance, increased only in NDO patients. CONCLUSIONS: The reduction in entropy during HD indicates impaired autonomic control in response to external perturbations. This state is partially reversed by the infusion of glucose with differences in central and peripheral responsiveness in DM and NDO patients.

Testosterone Deficiency and Nutritional Parameters as Predictors of All-Cause Mortality among Male Dialysis Patients.

Background: Chronic kidney disease (CKD) is associated with an accelerated risk of cardiovascular mortality. Hormonal and metabolic disorders in CKD may constitute novel risk factors. Our objective was to characterize and evaluate prognostic implications of circulating sex steroids and selected nutritional parameters in patients at different stages of CKD. Methods: Studied groups were composed of 78 men: 31 on hemodialysis (HD), 17 on peritoneal dialysis (PD), 30 with CKD stage G3-G4. Total testosterone (TT), dehydroepiandrosterone sulphate (DHEA-S), androstenedione, luteinizing hormone (LH), prolactin (PRL), and biochemical parameters were measured; Free testosterone (FT) was calculated. Results: The lowest TT and FT were observed in HD, the highest- in CKD (p = 0.006 for TT, p = 0.005 for FT). TT positively correlated with total cholesterol in HD (p = 0.012), FT negatively correlated with BMI in CKD (p = 0.023). During the 12 months, 9 patients died (5 in the HD, 4 in the PD group). The deceased group had significantly lower concentrations of albumin (p = 0.006) and prealbumin (p = 0.001), and a significantly higher concentration of androstenedione (p = 0.019) than the surviving group. In the group of men on dialysis, a serum TT concentration <2.55 ng/mL (Q1-first quartile) was associated with a 3.7-fold higher risk of death, although statistical significance was not achieved (p = 0.198). After analysis of the ROC curves, the FT level was the best prognostic marker in HD (AUC = 0.788; 95% CI: 0.581−0.996; p = 0.006) Conclusions: Total and free testosterone levels were lower in the HD group than in the CKD group. The nutritional status undoubtedly affects the survival of dialysis patients but also the concentrations of testosterone significantly contributes to further worsening the prognosis.

Left Ventricular Diastolic Dysfunction in Chronic Kidney Disease Patients Not Treated with Dialysis.

BACKGROUND: Left ventricular diastolic dysfunction (LVDD) is observed in the early stages of chronic kidney disease (CKD) and may lead to heart failure with preserved ejection fraction (HFpEF). The purpose of our study was to investigate the association between metabolic, nutritional and inflammatory parameters and LVDD in CKD and non-CKD patients. METHODS: Two groups of patients were recruited to the study: 93 men with CKD and eGFR lower than 60 mL/min/1.73 m2 and 40 men without kidney function decrease with eGFR ≥ 60 mL/min/1.73 m2. Transthoracic echocardiography was performed to evaluate the diastolic function of the left ventricle. Bioimpedance spectroscopy (BIS) was used to measure overhydration and lean body mass. We also measured the serum concentrations of albumin, glucose, haemoglobin A1c (HgbA1c), fibrinogen, C-reactive protein (CRP), tumor necrosis factor-alpha (TNF-alpha) and osteoprotegerin (OPG). RESULTS: We observed that elevated serum fibrinogen and glucose concentrations were associated with LVDD independently of CKD status. Serum fibrinogen concentrations increased with the advancement of LVDD. Low albumin concentrations in CKD were related with LVDD. In the control group, lower muscle mass presented as lean tissue index (LTI) and lean tissue mass (LTM), and overhydration were associated with LVDD. In the group of patients without kidney function decrease the OPG concentrations were significantly higher in those with LVDD, and they rose with the advancement of LVDD. CONCLUSIONS: Elevated inflammatory parameters, increased serum glucose concentrations and worse nutritional status are the states that may impair the diastolic function of the left ventricle in CKD and non-CKD patients. Serum OPG levels are elevated in patients without kidney function decrease and LVDD and its concentrations rise with the advancement of LVDD.

[Diagnostic difficulties of IgG4-related disease - a case report]. / Trudnosci diagnostyczne choroby zwiazanej z IgG4 ­ opis przypadku.

IgG4-related disease belongs to the group of immune-mediated diseases. It is a relatively new condition, classified in 2003. It is characterized by involvement of multiple organs, over time causing organ failure. The observed radiological changes are slow growing. As a rule, the course of the disease is subclinical, which means that the diagnosis is often made incidentally, retrospectively and many years after the onset of symptoms. In the initial stage of diagnosis, it is often confused with Sjögren's syndrome, systemic vasculitis, or neoplastic disorders. A CASE REPORT: We report the case of a female patient who was diagnosed with glomerulonephritis at the age of 34. The patient was qualified for renal replacement therapy with chronic hemodialyses in 2020 due to end-stage renal disease. In the same year, the patient was hospitalized for pneumonia of unknown etiology. After discharge from the hospital, the patient developed fever up to 39oC, chills, and pleural fluid. A suspicion of systemic disease was raised. Computed tomography of the chest showed enlargement of the anterior and middle mediastinum with increased densitization of adipose tissue and enlargement of mediastinal lymph nodes. Antibiotic therapy was introduced, followed by systemic corticosteroids, resulting in clinical improvement and remission of infiltrative lesions in radiological studies. The differential diagnosis excluded neoplastic lesions of the left lung, systemic connective tissue diseases, e.g. systemic lupus erythematosus and systemic vasculitis. Taking into account the whole clinical picture the suspicion of IgG4-related disease was raised. After the treatment, serum IgG4 level was found to be normal at 36.6 mg/ dl, anti-nuclear antibodies (ANA) were negative, rheumatoid factor was not elevated, complement component 4 (C4) was normal, complement component 3 (C3) decreased to 71 mg/dl (n: 90-180 mg/dl). Treatment with prednisone 10 mg/day was maintained. Histopathologic reanalysis of the renal biopsy (from a biopsy performed in 2012) was performed, which showed lymphocytic infiltration in the renal interstitium with segmental vascular loop sclerosis in the glomeruli and the presence of abundant granular IgG4 deposits in the glomerular mesangium by immunofluorescence. CONCLUSIONS: IgG4-related disease poses great diagnostic difficulty due to its non-specific and long-term course. Patients usually seek help with various specialists many years before the full picture of the disease is presented. It is poorly understood as it is a recently described condition (two decades ago). The classification criteria of IgG4 disease developed in 2019, the growing number of publications and recommendations being developed give hope for better understanding and more efficient diagnosis of the disease.