Goodpasture's Syndrome Research Articles

Autoimmune diagnostics in nephrology and rheumatology

Autoimmune diagnostics plays a central role in the detection of various acute and/or chronic diseases in both nephrology and rheumatology, which are associated with high morbidity and mortality if left untreated or not detected in time. Patients are threatened with significant limitations in everyday skills and quality of life due to loss of kidney function and dialysis, immobilizing and destructive joint processes or also significant damage of organ systems. In all of these autoimmune diseases, early diagnosis and treatment is of central importance for the further course and prognosis of disease.Antibodies play an essential role in the pathogenesis of autoimmune diseases. Antibodies are either directed against organ or tissue-specific antigens, such as in primary membranous glomerulonephritis or Goodpasture's syndrome, or they lead to a systemic disease such as systemic lupus erythematosus (SLE) or rheumatoid arthritis.Knowledge of the sensitivity and specificity of antibodies is crucial for the interpretation of antibody diagnostics results. Antibody detection can precede the clinical onset of the disease, and antibody titers often reflect disease activity. However, there are also false positive results. Detection of antibodies in the absence of disease symptoms often leads to uncertainty and unnecessary further diagnostics. Therefore, an unfounded "antibody screening" is not recommended.A rational antibody diagnostics is an integral part of the diagnostics and during treatment of nephrological and rheumatological diseases like glomerulonephrititis, pulmorenal syndrome, SLE and other collagenosis, thrombotic microangiopathy (HUS/TTP) and rheumatoid arthritis.

IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating anti-neutrophil cytoplasm antibodies (ANCA), where the diagnosis was established on kidney biopsy by detecting linear deposition of IgA along the GBM on immunofluorescence microscopy. Despite an intensive immunosuppressive regimen with plasmapheresis, steroids and oral cyclophosphamide, the disease progressed to end-stage renal failure and the patient was started on hemodialysis.

An atypical anti-GBM disease complicated by idiopathic nodular glomerulosclerosis: Case report.

Both atypical anti-glomerular basement membrane (anti-GBM) disease and idiopathic nodular glomerulosclerosis are rare diseases. We report a case of a 53-year-old non-diabetic male who presented with leg edema, nephritic range proteinuria, microscopic hematuria, and decreased renal function. The renal biopsy demonstrated membranoproliferative glomerulonephritis (MPGN) pattern of glomerular injury with focal crescent and segmental nodular glomerulosclerosis. The immunofluorescence studies showed intense linear IgG (IgG1 and IgG4) deposits along the GBM but negative serology. Electron microscopy demonstrated GBM thickening and fibrillar deposition. The presence of MPGN with crescents and the linear IgG along the GBM were consistent with a diagnosis of atypical ant-GBM disease. Superimposed nodular glomerulosclerosis was considered to be idiopathic by excluding other glomerular diseases characterized by fibrillar deposition and nodular glomerulosclerosis. Both diseases were found to have a strong causative association with patient's history of long-term heavy smoking. This unusual case with combination of atypical anti-GBM disease and idiopathic nodular glomerulosclerosis, has brought great challenge for the diagnosis and also made the clinical course highly complicated. This nodular glomerulosclerosis with anti-GBM-like glomerulonephritis may represent a distinct pattern of kidney injury observed in heavy smokers.

Prospects for the introduction of fodder species Artemisia into culture

In many desert communities, the basis of pasture ecosystems are species of the genus Artemisia, which are edifiers of plant communities in the vast territories of the Central Asia desert zone. Sagebrush pastures with a diverse set of plant species and life forms are used in desert animal husbandry all year round. Artemisia spp. is good fodder pastures plants. Green twigs contain 16.9% protein, 4.4% fat, 39% nitrogen-free extractives. Artemisia diffusa, А. turanica are common on desert graybrown and gray-earth soils with different granulometric composition, Artemisia halophila has a narrow ecological area confined to saline soils. The fodder mass of Artemisia spp. serves as the main type of sheep fodder and is especially appreciated in the autumn, winter and early spring periods. As a result of irrational use, the natural sagebrush pastures of deserts are degraded and need to restore their fodder productivity. All three wild–growing species – Artemisia turanica, Artemisia diffusa, Artemisia halophyla in culture conditions accelerate the passage of phenological phases in the process of ontogenesis. Individuals of species populations of sagebrush develop faster in cultural conditions than in natural sagebrush communities. In the culture conditions the process of ontogenesis, skeletal axes of the shrub are laid at the base of the maternal shoots in young individuals, in the second or third year of life, the tested species of sagebrush form a morphologically full-fledged life form of a semishrub with a feed productivity 3-5 times higher than the yield of natural pastures. In the conditions of Artemisia spp. culture, they grow well and quickly. Many individuals enter the fruiting season in the first year of life.

Nephrology: what's new in 2022

Severe cases of IGA nephropathy might benefit from corticosteroid therapy. Inflimidase may be a promising treatment of Goodpasture disease. SGLT2 inhibitors and acetazolamide act synergistically with loop diuretics in the treatment of acute cardiac failure. In hemodialysis, use of lung ultrasound to determine the ultrafiltration seems to decrease hospitalizations due to acute heart failure but does not reduce patient-centered outcomes. Icodextrin may mitigate the loss of ultrafiltration in PD patients who are carriers of the Aquaporin I promotor TT genotype. MICA-antibodies have an impact on the risk of graft rejection. Xenotransplantation may become a reality.

Anti-glomerular basement membrane disease: Treatment outcome of cyclophosphamide vs. rituximab induction therapy regimen.

To study the clinical profile of anti-glomerular basement membrane (anti-GBM) disease and its outcome with two different treatment regimens comprising either cyclophosphamide (CYC) or rituximab (RTX). A retrospective analysis of anti-GBM crescentic glomerulonephritis patients admitted to our hospital over 5 years. 14 patients were diagnosed with anti-GBM crescentic glomerulonephritis. The mean duration of symptoms was 3.6±1.9 weeks. All patients presented with rapidly progressive glomerulonephritis (RPGN). Five (35.7%) patients had concomitant urinary tract infection (UTI), 2 (14.3% had underlying type 2 diabetes mellitus, 5 (35.7%) patients also had positive anti-neutrophil cytoplasmic antibodies (ANCA), and 9 (64.3%) were dialysis-dependent at presentation. Four (28.6%) patients developed diffuse alveolar hemorrhage (DAH). All patients received baseline corticosteroids, and 7 (50%) patients also received plasmapheresis. Nine (64.3%) patients were treated with CYC, and 3 (21.4%) patients received RTX. In the CYC arm, 2 (28.6%) patients died, 3 had end-stage kidney disease (ESKD) at 3 months, and 2 had chronic kidney disease (CKD) stage III at 3 months of follow-up. Two patients were lost to follow-up. In the RTX arm, all 3 patients survived with no incidence of DAH, 1patient each had ESKD and CKD stage III, and 1 dialysis-dependent patient achieved normal kidney function at the end of 3 months. Most patients presented late with dialysis-dependent renal failure, and many had concomitant UTI. Concomitant infection causes diagnostic confusion with RPGN and DAH, which delays diagnosis and treatment. RTX as an alternative to CYC in addition to baseline corticosteroids and/or plasmapheresis and is associated with favorable outcomes.

Anti-glomerular basement membrane disease developing 3years after the development of Sweet syndrome and 1year after the development of anti-neutrophil cytoplasmic antibody-associated vasculitis: a case report.

A 73-year-old Japanese woman, with a history of Sweet syndrome diagnosed 3years earlier and anti-myeloperoxidase (MPO) antibody anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis diagnosed 1year earlier, presented with an episode of rapidly progressive glomerulonephritis (RPGN) with anti-glomerular basement membrane (GBM) disease. At the time of diagnosis of the ANCA-associated vasculitis 1 year earlier, serological testing yielded a negative result for anti-GBM antibody. However, at the present visit, serology for anti-MPO antibody was negative, while that for anti-GBM antibody was positive. This is the first report of anti-GBM disease developing sequentially after Sweet syndrome and ANCA-associated vasculitis. This case may provide clues to the potential immunological links among these three distinct conditions.

Pulmonary renal syndrome: a clinical review.

The term "pulmonary renal syndrome" describes a clinical syndrome which is characterised by the presence of both diffuse alveolar haemorrhage and glomerulonephritis. It encompasses a group of diseases with distinctive clinical and radiological manifestations, as well as different pathophysiological processes. The most common diseases implicated are anti-neutrophil cytoplasm antibodies (ANCA)-positive small vessel vasculitis and anti-glomerular basement membrane (anti-GBM) disease. Prompt recognition is required as respiratory failure and end-stage renal failure can rapidly occur. Treatment includes a combination of glucocorticoids, immunosuppression, plasmapheresis and supportive measures. The use of targeted treatments has significantly reduced mortality. Thus, an understanding of pulmonary renal syndrome is essential for the respiratory physician.

A case report of atypical anti-glomerular basement membrane disease

BackgroundAnti-glomerular basement membrane (anti-GBM) disease is characterized by crescentic necrotizing glomerulonephritis, with linear deposits of immunoglobulin G (IgG) in the GBM. Classic anti-GBM disease is clinically associated with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Some patients have a better renal prognosis and milder symptoms than those with classic anti-GBM disease, which is termed atypical anti-GBM disease.Case presentationA 43-year-old Japanese woman was admitted to our hospital complaining of hematuria that had persisted for more than one month. Serological examination revealed negativity for anti-nuclear, anti-neutrophilic cytoplasmic, and anti-GBM antibodies. However, renal biopsy showed cellular crescents. Immunofluorescence revealed strong diffuse linear capillary loop staining for IgG. An indirect immunofluorescence antibody method was performed by applying the patient serum to normal kidney tissue to confirm the presence of autoantibodies binding to the GBM. Using this method, anti-GBM antibodies were detected. The patient was treated with high-dose steroids, cyclophosphamide, and plasma exchange. Aggressive treatment resolved proteinuria and hematuria and improved renal function.ConclusionsRenal biopsy is crucial in the diagnosis of anti-GBM disease, especially when serological tests are negative. Accurately identifying the presence of anti-GBM disease is important to initiate optimal treatment.

Clinical and pathological features of anti-glomerular basement membrane disease associated with membranous nephropathy: an observational study

To investigate the clinical manifestations, pathological features, pathogenesis, treatment, and prognosis of anti-glomerular basement membrane (anti-GBM) disease with membranous nephropathy (MN). Seven patients with anti-GBM disease and concurrent MN were enrolled in this study. Control subjects included 13 patients with anti-GBM glomerulonephritis (GN) and 6 with anti-GBM disease and concurrent anti-neutrophil cytoplasmic antibodies-associated disease (anti-GBM + ANCA). Laboratory tests and pathological information were analyzed before immunosuppressive therapy or plasmapheresis administration. Prognosis was assessed in continuous follow-up. In the anti-GBM + MN group, 28.57% of patients exhibited acute kidney disease, lower than that in the anti-GBM GN group (84.62%, p = .022). None of the anti-GBM + MN or + ANCA patients exhibited hemoptysis, but 15.4% of anti-GBM GN patients did, with no significant difference (p = .720). Only 14.3% of anti-GBM + MN patients had crescentic GN. The proportion of necrosis averaged 29.0% in the anti-GBM + MN group. Survival curve analysis revealed that renal outcomes in the anti-GBM + MN group were better than those in the anti-GBM GN group (p = .019). Patients with both anti-GBM disease and MN showed atypical anti-GBM GN. They had a lower proportion of glomerular crescents and a better renal function prognosis than patients with classical anti-GBM GN. To improve renal recovery, early identification and treatment of anti-GBM disease associated with MN is needed.

Feeding tannins to dairy cows in different seasons improves the oxidative status of blood plasma and the antioxidant capacity of cheese.

The aim of the present study was to assess the dietary supplementation of tannins to grazing dairy cows in 2 seasons characterized by a good quality pasture (spring) or a poor-quality pasture (summer). The effects of dietary tannins were assessed on plasma antioxidant status and cytokines profile and on the antioxidant properties of cheese and cheese in vitro digestates. Fourteen lactating dairy cows were divided into 2 homogeneous groups (n = 7): a control group (CON), and an experimental group (TAN) receiving 150 g/head per day of tannins supplementation. The experiment was performed twice, in spring and in summer. The animals were free to graze on spontaneous pasture (spring) or on dry stubble (summer). Blood was sampled at the beginning (d 0), at the midpoint (d 11), and at the end (d 22) of the trial. Individual cheese was produced before the beginning (d -1) and at the end (d 22) of the trial from the milk collected from each cow. On blood plasma, the reactive oxygen metabolites (ROM), biological antioxidant potential (BAP), nonesterified fatty acids quantification, and cytokines profile in terms of IL-10, IL-8, IL-1β, and IFN-γ were determined. Data on ROM demonstrated that tannins supplementation lowered oxidative stress both in spring and in summer. Accordingly, TAN diet increased BAP levels compared with the CON during summer trial. Thus, feeding tannins resulted in lower ratio between ROM and BAP (oxidative stress index) in both spring and summer. Cytokines' profile showed lower IL-1β values in TAN group at d 22 during spring season, with a concomitant higher IL-10 level, during summer season. Moreover, TAN group had a lower level of IFN-γ in plasma than CON group, both in spring and in summer. On cheese samples, the in vitro digestion was performed and on cheese and cheese digestates (gastric and intestinal digestate) the free radical scavenging antioxidant activity was evaluated. The intestinal digestate fraction registered the highest antioxidant activity compared with cheese and gastric digestate, in both spring and summer seasons. Furthermore, an improvement of the antioxidant property of cheese and cheese digestates was found. Present data demonstrated that tannins supplementation contributed to reduce the oxidative stress of lactating dairy cows and showed an increase of anti-inflammatory cytokines ratio.

Contrasting Fecal Methanogenic and Bacterial Profiles of Organic Dairy Cows Located in Northwest Washington Receiving Either a Mixed Diet of Pasture and TMR or Solely TMR.

Simple SummaryMethane is a by-product of the microbial fermentation process from a group of archaea known as methanogens. Ruminants harbor methanogens as a component of their normal gastrointestinal microbiota. In this study, we assessed the fecal microbiome of organic dairy cows across different time points receiving a mixed diet of pasture and TMR or TMR only. Our aim was to compare the archaea and bacterial fecal microbial structure, diversity and their interactions across diets. Cluster analysis based on the cows’ fecal microbial community identified four clusters. We found little difference in the relative abundance of methanogens across clusters. However, there was evidence of differences in diversity between pasture associated communities and those associated with TMR only. The cluster associated with cows receiving high-quality pasture and TMR, had higher diversity and a less robust co-occurrence network than those in TMR only or lower-quality pasture communities. The overall good pasture and TMR quality, combined with the organic allowance for feeding high levels of TMR, might have contributed to the lack of differences in the fecal archaeal community. The development of novel strategies that are independent of pasture management could have a greater impact in helping to reduce enteric CH4 emissions on organic dairies.Currently, little is known regarding fecal microbial populations and their associations with methanogenic archaea in pasture-based dairy cattle. In this study, we assessed the fecal microbiome of organic dairy cows across different time points receiving a mixed diet of pasture and total mixed ration (TMR) or TMR only. We hypothesized that the fecal methanogenic community, as well as co-occurrence patterns with bacteria, change across diets. To test these hypotheses, we analyzed TMR and pasture samples, as well as the V3-V4 region of 16S rRNA of fecal samples collected over the course of a one-year study period from 209 cows located on an organic dairy in Northwest Washington. The inherent variability in pasture quality, quantity, availability, and animal preference can lead to diverse dietary intakes. Therefore, we conducted a k-means clustering analysis to identify samples from cows that were associated with either a pasture-based diet or a solely TMR diet. A total of 4 clusters were identified. Clusters 1 and 3 were mainly associated with samples primarily collected from cows with access to pasture of varying quality and TMR, cluster 2 was formed by samples from cows receiving only TMR, and cluster 4 was a mix of samples from cows receiving high-quality pasture and TMR or TMR only. Interestingly, we found little difference in the relative abundance of methanogens between the community clusters. There was evidence of differences in diversity between pasture associated bacterial communities and those associated with TMR. Cluster 4 had higher diversity and a less robust co-occurrence network based on Spearman correlations than communities representing TMR only or lower-quality pasture samples. These findings indicate that varied bacterial communities are correlated with the metabolic characteristics of different diets. The overall good pasture and TMR quality in this study, combined with the organic allowance for feeding high levels of TMR even during the grazing season, might have contributed to the lack of differences in the fecal archaeal community from samples associated with a mixed pasture and TMR diet, and a TMR only diet. Mitigation strategies to decrease methane emissions such as increasing concentrate to forage ratio, decreasing pasture maturity and adopting grazing systems targeting high quality pasture have been shown to be efficient for pasture-based systems. However, the allowance for organic dairy producers to provide up to an average of 70% of a ruminant’s dry matter demand from dry matter fed (e.g., TMR), suggests that reducing enteric methane emissions may require the development of novel dietary strategies independent of pasture management.

A RARE CASE OF SERONEGATIVE PULMONARY RENAL SYNDROME

A RARE CASE OF SERONEGATIVE PULMONARY RENAL SYNDROME

S760 Epidemiology of Glomerulonephritis as an Extraintestinal Manifestation of Inflammatory Bowel Disease: A Large Population-Based Study

Introduction: Inflammatory bowel disease (IBD), including both Crohn’s disease (CD) and Ulcerative colitis (UC), are known to cause many extraintestinal manifestations. There is limited data describing the association between IBD and renal manifestations. This study provides epidemiologic data to further understand the association between IBD and glomerulonephritis (GN). Methods: The aim of this study was to investigate if IBD is associated with GN. Data was collected from a commercial database (Explorys Inc, Cleveland, OH), an aggregate of EHR data from 27 integrated healthcare systems in the US between 1/2017-1/2022. We identified patients with CD and UC based on Systemized Nomenclature of Medicine – Clinical Terms. We compared the prevalence of GN at least 30 days post-CD or post-UC diagnosis to a control cohort without CD or UC. We excluded patients with a diagnostic predisposition for development of GN (Table). Results: Of the 34,063,760 patients, we identified 149,630 cases of CD and 127,300 cases of UC. There were 310 and 260 cases of glomerulonephritis at least 30 days after diagnosis of CD and UC, respectively. Overall prevalence of glomerulonephritis for CD and UC was 207/100000 persons and 203/100000 persons, whereas for the cohort without CD and UC was 72/100000 persons and 72/100000 persons. Prevalence ratios (PR) of developing glomerulonephritis after at least 30 days of a CD and UC diagnosis were 2.88 (95% CI, 2.57-3.22, p< 0.001) and 2.83 (95% CI, 2.51-3.20, p< 0.001), respectively. Figure shows prevalence and PR in CD and UC sub-categorized by demographics. Membranous GN was the most prevalent (CD 90, UC 90), followed by proliferative (CD 50, UC 50), mesangiocapillary (CD 30, UC20), diffuse (CD 10, UC 0) and focal membranoproliferative (CD 10, UC 10). Conclusion: In this large population-based study, glomerulonephritis was significantly more prevalent in patients with both CD and UC compared to those without IBD. Further studies are needed to understand the pathophysiological mechanism underlying renal involvement.Figure 1.: Prevalence and prevalence ratios of glomerulonephritis after at least 30 days post-CD and post-UC diagnosis. Table 1. - Diagnoses excluded prior to analysis Excluded diagnoses Systemic lupus erythematosus Goodpasture's syndrome Hereditary nephritis Post-infectious GN Granulomatosis with polyangiitis Allergic grandulomatosis angiitis Alport syndrome Human immunodeficiency virus Hepatitic B virus Hepatitis C virus Nephrotic syndrome with membranoproliferative glomerulonephritis Primary pauci-immune necrotizing and crescentic glomerulonephritis Berger's immunoglobulin A or immunoglobulin G nephropathy

Anti–glomerular basement membrane disease with rapidly progressive glomerulonephritis and hemorrhagic alveolitis in pediatric patients (reviev of literature)

The article is devoted to controversial issues of autoimmune small vessel vasculitis with kidney and lung damage. Modern data on the features of pathogenesis, clinical manifestation, treatment, outcome, and prognosis of the disease with AT to GBM have presented: an idiopathic disease with AT of class IgG (IgG1 and IgG3) against non-collagen domain-1 (NC1) α3-chain ofcollagen type IV GBM (Goodpasture autoantigen); with the simultaneous increase in AT to GBM and ANCA; "de novo" diseases with AT to the α5(IV) and α3(IV) chains of GBM type IV collagen in renal graft with Alport syndrome in pediatric patients.

Treatment and Prevention of Anti-Glomerular Basement Membrane Disease a Tertiary Care Hospital Study

Patients with anti-glomerular basement membrane (anti-GBM) disease have Type IV collagen antigens in their glomerular and alveolar basement membranes. GN or alveolar bleeding develops quickly. Genetically vulnerable people may be activated by environmental factors. There isn't enough evidence to indicate a causal relationship between SARS- cov-2 and COVID-19. One in ten crescentic kidney disease patients has anti-GBM sickness. Early identification of circulating antibodies, increased awareness of rare and challenging clinical manifestations, and immunosuppressive and plasma exchange therapy have improved patients' prognoses. Anti-GBM disease is rare, but new medications and treatments are coming.

Consequence of Environmental Change on the Animal’s Health and Productivity: A Brief Review

The effects of climate change, especially global warming, can greatly affect the production performance and health of animals around the world. The national average temperature has increased by 1°C since 1970. Most livestock owners in the country feel that weather change is affecting farm animal production and health. The main impacts of weather change on animal production include feed shortages, water shortages, reduced livestock genomic resources, decreased productivity, and reduced mature weight and/or longer time to reach mature weight based on their significance. High temperatures resulting from environmental alteration may increase the level of development of few pathogens or parasites that found one or more life cycle levels outside the mammalian host. Besides, the spatial disposition and visibility of pasture and water are largely depending on the pattern and visibility of rainfall. Food and water shortages bestow to deduced livestock abundance and reproductive execution. These include slow growth rate of animals, loss of body condition, decreased milk yield, and poor reproductive performance of mature animals. Drought bulls that are debilitated and in poor physical condition cannot provide sufficient drought energy for plowing and thus hinder crop cultivation. Bush encroachment, as well as population pressure, leads to reduced availability of good pastures thus environmental change will have far-reaching consequences for animal yield and health, especially in vulnerable parts of nature where it is essential for nutrients and maintenance. Once more, the environmental change affects farm animal health through various mechanisms; which are effects on parasites, effects on hosts, and vectors, such as alters in precipitation and temperature schemes that can influence both the placement and ample of disease careers; and effects on micropaleontology, such as alteration of transmission levels between hosts. This study has focused to investigate the inherent impacts of environmental alteration on livestock health and productivity.

The presentation, etiologies, pathophysiology, and treatment of pulmonary renal syndrome: A review of the literature

Pulmonary renal syndrome (PRS) is a constellation of different disorders that cause both rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. While antineutrophil cytoplasmic antibody associated vasculitis and anti-glomerular basement membrane disease are the predominant causes of PRS, numerous other mechanisms have been shown to cause this syndrome, including thrombotic microangiopathies, drug exposures, and infections, among others. This syndrome has high morbidity and mortality, and early diagnosis and treatment is imperative to improve outcomes. Treatment generally involves glucocorticoids and immunosuppressive agents, but treatment targeted to the underlying disorder can improve outcomes and mitigate side effects. Familiarity with the wide range of possible causes of PRS can aid the clinician in workup, diagnosis and early initiation of treatment. This review provides a summary of the clinical presentation, etiologies, pathophysiology, and treatment of PRS.

Rituximab for the treatment of refractory anti-glomerular basement membrane disease

Background Anti-glomerular basement membrane (anti-GBM) disease is a rare but severe autoantibody-mediated immune disorder. The typical clinical presentation includes rapidly progressive glomerulonephritis and often concurrent pulmonary hemorrhage. The present study is aimed to investigate the therapeutic effects of rituximab either used alone or with other immunosuppressants. Methods Eight patients diagnosed with anti-GBM disease and treated with rituximab from 2014 to 2020 were retrospectively reviewed. Results Eight patients included 5 males and 3 females with a median age of 58.5 years. They all presented severe kidney injuries and 1 patient had lung hemorrhage. At diagnosis, the median of serum creatinine was 246 µmol/L (ranging from 91 to 850 µmol/L), with 3 patients requiring dialysis. All of them received corticosteroids and plasmapheresis. Rituximab was given as either standard four weekly doses or one pulse ranging from 100 to 600 mg. After a median follow-up of 34.5 months, kidney function was partially recovered or stabilized in 5/8 (62.5%) patients, free of dialysis. Anti-GBM antibodies remained undetected in all patients during follow-up. No severe adverse effect associated with rituximab was observed. Conclusion Rituximab may be an alternative therapy in the treatment of patient with severe or refractory anti-GBM disease.

Influence of liquids for electronic cigarettes on the human respiratory system. Clinical observation of a patient with EVALI

Aim. Demonstration of clinical observation of EVALI from the personal practice of the authors.Materials and methods. A brief review of the literature on the effects of e-liquids on the human respiratory system is presented. Clinical observation of this pathology is given.Results. Patient H., 19 years old, was admitted to the Thoracic Surgery Department with complaints of shortness of breath, cough with hemoptysis. On computer tomography in lung parenchyma, infiltrates are found in all fields with extensive ground glass areas. Fibrobronchoscopy revealed in the lumen of the bronchi a small amount of hemorrhagic “crusts” and hemorrhagic exudate, catarrhal-hemorrhagic endobronchitis. The patient is a fan of electronic cigarettes, which was not immediately paid attention to. A differential diagnosis was made with Goodpasture's syndrome, idiopathic pulmonary hemosiderosis, systemic lupus erythematosus with lung involvement, and ANCA-associated vasculitis. Since there was nephropathy and there were negative immunological findings of other diseases, a diagnosis of Goodpasture's syndrome was made. A kidney biopsy was not performed. The rapid positive dynamics from hormonal therapy and the absence of relapses and progression raised doubts about this diagnosis. After the disease, the patient refused to take electronic cigarettes. The anamnesis was again addressed, where there were indications of the abuse of electronic cigarettes, after frequent use of which a detailed clinical picture of the disease appeared, the diagnosis of Goodpasture's syndrome was withdrawn, lung damage was regarded as a manifestation of EVALI.Conclusion. Confirmation of the negative impact of electronic smoking systems is the described clinical case, indicating lung damage in humans and difficulties in the differential diagnosis of EVALI syndrome.