Goodpasture's Syndrome Research Articles

Pneumorenal Syndrome Complicating ANCA-Negative Pauci-Immune Vasculitis Associated with Atypical Mycobacteriosis: Case Report

Among the forms of pneumorenal syndrome (PRS), one of the most urgent is characterized by the combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis (RPGN). In 90% of cases, it is the manifestation of an autoimmune disease, such as pauci-immune vasculitis with anti-neutrophil cytoplasmic antibodies (ANCA) or Goodpasture's disease with the presence of antibodies directed against the basement membrane of the glomerular and alveolar capillaries (anti-GBM). These antibodies are absent in approximately 10% of cases with histological confirmation in the context of rare clinical situations. We report a case of a 66-year-old patient, with a history of alcoholism and active smoking, who was admitted to the hospital for respiratory distress with hemoptysis. The admission assessment showed severe renal failure requiring hemodialysis upon admission. ANCA and anti-GBM, as well as all other autoantibodies were negative. A renal biopsy revealed pauci-immune extra-capillary glomerulonephritis with the majority of chronic lesions. The orientation of the diagnostic approach in search of other causes of pauci-immune vasculitis with negative antibodies has been initiated. He was treated with glucocorticoids in combination with cyclophosphamide. The initial evolution was marked by the improvement of respiratory symptoms and a less considerable recovery of renal function. PRS can develop without the presence of ANCA or other autoantibodies. The diagnosis is then made by histology. Renal damage, such as extra-capillary glomerulonephritis (GN), is more severe and has a very poor prognosis with a high risk of mortality. The absence of ANCA should never delay diagnosis and treatment.

Three Diseases Mediated by Different Immunopathologic Mechanisms-ANCA-Associated Vasculitis, Anti-Glomerular Basement Membrane Disease, and Immune Complex-Mediated Glomerulonephritis-A Common Clinical and Histopathologic Picture: Rapidly Progressive Crescentic Glomerulonephritis.

Immune mechanisms play an important role in the pathogenesis of glomerulonephritis (GN), with autoimmunity being the main underlying pathogenetic process of both primary and secondary GN. We present three autoimmune diseases mediated by different autoimmune mechanisms: glomerulonephritis in vasculitis mediated by anti-neutrophil cytoplasmic antibodies (ANCAs), glomerulonephritis mediated by anti-glomerular basement membrane antibodies (anti-GBM antibodies), and immune complex-mediated glomerulonephritis. Some of these diseases represent a common clinical and histopathologic scenario, namely rapidly progressive crescentic glomerulonephritis. This is a severe illness requiring complex therapy, with the main role being played by therapy aimed at targeting immune mechanisms. In the absence of immune therapy, the crescents, the characteristic histopathologic lesions of this common presentation, progress toward fibrosis, which is accompanied by end-stage renal disease (ESRD). The fact that three diseases mediated by different immunopathologic mechanisms have a common clinical and histopathologic picture reveals the complexity of the relationship between immunopathologic mechanisms and their clinical expression. Whereas most glomerular diseases progress by a slow process of sclerosis and fibrosis, the glomerular diseases accompanied by glomerular crescent formation can progress, if untreated, in a couple of months into whole-nephron glomerulosclerosis and fibrosis. The outcome of different immune processes in a common clinical and histopathologic phenotype reveals the complexity of the relationship of the kidney with the immune system. The aim of this review is to present different immune processes that lead to a common clinical and histopathologic phenotype, such as rapidly progressive crescentic glomerulonephritis.

Goodpasture Syndrome with Neurological Condition Associated with anti-MBG Ab, Negative ANCA: Case Report

Goodpasture syndrome is a rare immunologically based clinical entity that is characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage, and the presence of circulating autoantibodies against the glomerular basement membrane (GBM). Concomitant central nervous system (CNS) manifestations are an unusual presentation of GSP in the absence of ANCA. We present a case of atypical course of the disease in a 48-year-old female patient who started with the presence of hemoptoic sputums and manifestations of uremic syndrome in the presence of high titres of glomerular basement anti membrane antibodies (Ac-anti-MBG) and negative ANCA, with a report of diffuse active extracapillary proliferative glomerulonephritis biopsy, of the pauciimmune type. After immunosuppressive treatment and plasmapheresis, the patient presented decreased anti-MBG Ab titres, as well as improvement of symptoms, however without recovery of renal function on hemodialysis, after 2 months he presents clonic-tonic seizures crisis, we conclude that PGS with neurological involvement is extremely rare, especially with negative ANCAs.

Botanical and turf management evaluation in goat pastures established from Pannonian natural and abandoned arable land

In animal husbandry, there is not a lot of available research on grazing by goats on grassland with different features. Based on this, the following research questions have been formed: What effect does grazing by goats have on different vegetation? How does vegetation react to grazing by goats under different grazing pressures? How do goats change the composition of vegetation in abandoned areas over a period of a few years? Which grasslands are the most useful for turf management? What grazing pressure is necessary to maintain natural vegetation? The differences are important to make grazing by goats efficient, so that goat farmers can apply grazing pressures that optimise the condition of the area in order to avoid degradation. To respond the questions, three goat pastures was studied in Somogy County, Hungary near Kaposdada and Kaposszerdahely. Different traits were used to evaluate the vegetation of these habitats. I: In Kaposszerdahely, where an abandoned arable field was converted into a goat pasture which is a cultivated pasture. II: In Kaposdada where there was natural grassland, which had low grazing pressure (1 animal/ha). III: In Kaposdada where there was overgrazing area (4 animals/ha). The studies were carried out for 4 years: coenological surveys were conducted in June 2015–2018 in 2 × 2 m quadrats. The vegetation of the areas was assessed in terms of turf management and nature conservation categories. Biomass investigations were applied in June and in September annually. In parallel with coenological recordings, 2 × 2 m. The forage values of various lawns were calculated by Balázs method. Based on the results, grazing by goats had a positive effect on sustaining the forage value of grassland in the typical Pannonian sample area. All sample areas had a high proportion of weeds and disturbance tolerant species, which is characteristic of this vegetation type. However, if we do not consider the nature conservation value and we would consider the grazing value of the species, the area was valuable for turf management, the proportion of grasses was significant, papilionaceous also occurred, although not in high proportions, and even the proportion of herbs was significant. The sample area I developed a semi-natural condition during the study period and overall, after 7 years. Also, in the case of forage values, the semi-natural area was a good pasture with a high animal carrying capacity. In the goat pasture, woody and shrub species were minimal or missed, confirming that these areas should be grazed by other animals, including sheep, based on their animal carrying capacity.

Extreme Fatigue as the Only Complaint in Double-Positive Anti-Glomerular Basement Membrane (GBM) and ANCA Disease: An Unusual Presentation

Extreme Fatigue as the Only Complaint in Double-Positive Anti-Glomerular Basement Membrane (GBM) and ANCA Disease: An Unusual Presentation

Atypical Presentation of Atypical Anti-Glomerular Basement Membrane Disease

Atypical Presentation of Atypical Anti-Glomerular Basement Membrane Disease

Anti-Glomerular Basement Membrane Disease Overlap with Pauci-Immune Glomerulonephritis: Temporal Concurrence at a Single Center

Anti-Glomerular Basement Membrane Disease Overlap with Pauci-Immune Glomerulonephritis: Temporal Concurrence at a Single Center

Prevalence and Outcomes of Anti-Glomerular Basement Membrane Disease with and Without Secondary Glomerular Pathologies

Prevalence and Outcomes of Anti-Glomerular Basement Membrane Disease with and Without Secondary Glomerular Pathologies

Prediction-Related Hint, Result and Effect of Anti-Neutrophil Cytoplasmic Autoantibodies with Myeloperoxidase Level of Detail in Anti-Glomerular Basement Membrane Disease

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The The effect of feeding on rapeseed and bee bread plants on the biological activity and productivity of honey bee colonies Apis mellifera

The field study was conducted during the agricultural season 2022-2023 in three locations. The first is the apiary of the College of Agriculture and Forestry, in which the bee sects were feeding and resting on the natural wild plants and bushes present in the apiary and the surrounding areas. The second location was in one of the agricultural fields in the Rashidieh area, in which it was planted. Seeds of the bee bread plant Borago officinalis at a rate of one dunam. The third site was in one of the agricultural fields of the Al-Mazra'i neighborhood of Nineveh Governorate, in which rapeseed Brassica napus was planted, at a rate of one dunam as well. The results of the study showed that the treatment of bee bread (the second site) on 1/10 was superior in recording the highest bee density, worker brood, pollen grain area and mature honey area, which was positively reflected in the amount of honey produced, with averages of 17.66 bees, 785.66 inches2, 302.17 inches2, and 803 inches. 33 inches2 and 9.08 kg / colony, respectively, and the treatment of rapeseed plants (the third site) recorded satisfactory and good results in which it outperformed the comparison treatment with averages of 14.42 bees, 596.66 inches2, 257.66 inches2, 760.33 inches2, and 7. 63 kg / colony, respectively, while this percentage decreased in the comparison treatment (first site) on 11/11, which recorded the lowest averages of 5.17 bees, 341.33 inches2, 40.66 inches2, 118.33 inches2, and 2.54 kg. Respectively, the results confirmed the necessity of cultivating bee bread and rapeseed plants to provide beds and a good pasture of nectar and pollen through which bee colonies can cross the winter stage and obtain high bee density and good storage of honey and pollen.

Age- and time-dependent increases in incident anti-glomerular basement membrane disease: a nationwide cohort study.

Epidemiologic assessments of anti-glomerular basement membrane (GBM) disease have been challenging due to its rare occurrence. We examined changes in the incidence and outcomes from 1998 to 2018 using nationwide healthcare registries. All patients with incident anti-GBM disease were identified using the International Classification of Diseases, 10th Revision code DM31.0A. Controls were matched 4:1 on birthyear and sex using exposure density sampling. Log link regression adjusted for time, age and sex was applied to model survival. We identified 97 patients with incident anti-GBM disease, corresponding to an incidence of 0.91cases/million/year [standard deviation (SD) 0.6]. The incidence increased over time [1998-2004: 0.50 (SD 0.2), 2005-2011: 0.80 (SD 0.4), 2012-2018: 1.4 (SD 0.5); P=.02] and with age [0.76 (SD 0.4), 1.5 (SD 1.04) and 4.9 (SD 2.6) for patients <45, 45-75 and >75years]. The median age was 56years (interquartile range 46) and 51.6% were female. Dialysis was required in 58.4%, 61.9% and 62.9% of patients at day 30, 180 and 360, respectively. The 1-year kidney survival probability was 0.38 (SD 0.05) and exhibited time-dependent changes [1998-2004: 0.47 (SD 0.13), 2005-2011: 0.16 (SD 0.07), 2012-2018: 0.46 (SD 0.07); P=.035]. The 5-year mortality was 26.8% and mortality remained stable over time (P=.228). The risk of death was greater than that of the matched background population {absolute risk ratio [ARR] 5.27 [confidence interval (CI) 2.45-11.3], P<.001}, however, it was comparable to that of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) requiring renal dialysis at presentation [ARR 0.82 (CI 0.48-1.41), P=.50]. The incidence of anti-GBM disease increased over time, possibly related to temporal demographic changes. Mortality remained high and was comparable with an age- and sex-matched cohort of dialysis-dependent AAV patients.

Clinicopathological Characteristics of Anti-Glomerular Basement Membrane Disease with Atypical Features

Clinicopathological Characteristics of Anti-Glomerular Basement Membrane Disease with Atypical Features

An intensive care case of Goodpasture's syndrome

A case report of a rare disease associated with antibodies to the basement membrane of the glomeruli of the kidneys (Goodpasture's syndrome) in a young woman is presented. The case is characterized by a typical course of the disease with a high titer of antibodies to the basement membrane of the glomeruli, kidney damage with the formation of terminal renal failure and infiltrative lung damage. The starting point for the development of the disease was probably massive tissue damage after an intense long massage. The diagnosis was confirmed clinically and by immunological data. The patient underwent intensive therapy, including the use of genetic engineering therapy with the subsequent development of covid-19 infection, effectively treated with traditional methods. During the year of observation, stabilization of the autoimmune process was achieved against the background of combination therapy. The difficulty of curing patients with pneumo-renal autoimmune damage and the possibility of achieving a positive course of the disease on the background of multicomponent intensive therapy are emphasized.

Anti-Glomerular Basement Membrane Disease in a Six-year-old Child: A Rare Presentation

Antiglomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis caused by auto-antibodies targeting auto-antigen expressed in basement membranes of capillary beds in lungs and kidneys. A six-year-old male presented with fever, cough, and generalized weakness for three days with respiratory distress for one day. The child had three prior admissions with similar complaints and was treated as a case of lower respiratory tract infection with severe iron deficiency anemia in the past. Multiple packed red cell transfusions were required in these past admissions. In this current admission, he had tachycardia, tachypnea, severe pallor, right-sided lung crepitations &amp; bronchial breath sounds. The hemogram showed severe anemia and leukocytosis. Urine examination showed mild proteinuria and microscopic hematuria. Chest X-ray (current admission) showed right middle and lower zone consolidation. HRCT of the chest revealed an interstitial pattern. A lung biopsy showed pulmonary hemorrhage and positive Prussian blue staining. Raised anti-GBM titers confirmed diagnosis of anti-GBM disease. Renal biopsy revealed normal glomerular morphology on histopathology; with immune fluorescence showing linear positivity for IgG (3+), Kappa (3+), and Lambda (3+). Electron microscopy reported mild effacement of visceral epithelial foot processes and subepithelial/intramembranous electron-dense deposits. He was treated with intravenous methylprednisone for 5 days followed by oral prednisolone for one month, which was thereafter tapered to a lower maintenance dose for 18 months. The child received six cycles of intravenous cyclophosphamide along with a daily low dose of steroids. Currently, the child is asymptomatic after one year of follow-up with seroconversion (antiGBM titers decreased) and normalization of the chest radiograph.

Unleashing the power of complement activation: unraveling renal damage in human anti-glomerular basement membrane disease.

Anti-glomerular basement membrane (GBM) disease is a rare but life-threatening autoimmune disorder characterized by rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Renal biopsies of anti-GBM patients predominantly show linear deposition of IgG and complement component 3 (C3), indicating a close association between antigen-antibody reactions and subsequent complement activation in the pathogenesis of the disease. All three major pathways of complement activation, including the classical, lectin, and alternative pathways, are involved in human anti-GBM disease. Several complement factors, such as C3, C5b-9, and factor B, show a positive correlation with the severity of the renal injury and act as risk factors for renal outcomes. Furthermore, compared to patients with single positivity for anti-GBM antibodies, individuals who are double-seropositive for anti-neutrophil cytoplasmic antibody (ANCA) and anti-GBM antibodies exhibit a unique clinical phenotype that lies between ANCA-associated vasculitis (AAV) and anti-GBM disease. Complement activation may serve as a potential "bridge" for triggering both AAV and anti-GBM conditions. The aim of this article is to provide a comprehensive review of the latest clinical evidence regarding the role of complement activation in anti-GBM disease. Furthermore, potential therapeutic strategies targeting complement components and associated precautions are discussed, to establish a theoretical basis for complement-targeted therapies.

NETosis: an emerging therapeutic target in renal diseases.

Neutrophil extracellular traps (NETs) are web-like structures composed of nuclear and granular components. The primary role of NETS is to prevent the dissemination of microbes and facilitate their elimination. However, this process is accompanied by collateral proinflammatory adverse effects when the NET release becomes uncontrollable, or clearance is impaired. Although NET-induced organ damage is conducted primarily and indirectly via immune complexes and the subsequent release of cytokines, their direct effects on cells are also remarkable. NETosis plays a critical pathogenic role in several renal disorders, such as the early phase of acute tubular necrosis, anti-neutrophil cytoplasmic antibody-mediated renal vasculitis, lupus nephritis, thrombotic microangiopathies, anti-glomerular basement membrane disease, and diabetic nephropathy. Their substantial contribution in the course of these disorders makes them a desirable target in the therapeutic armamentarium. This article gives an in-depth review of the heterogeneous pathogenesis and physiological regulations of NETosis and its pivotal role in renal diseases. Based on the pathogenesis, the article also outlines the current therapeutic options and possible molecular targets in the treatment of NET-related renal disorders. We carried out thorough literature research published in PubMed and Google Scholar, including a comprehensive review and analysis of the classification, pathomechanisms, and a broad spectrum of NET-related kidney disorders. NETosis plays a pivotal role in certain renal diseases. It initiates and maintains inflammatory and autoimmune disorders, thus making it a desirable target for improving patient and renal outcomes. Better understanding and clinical translation of the pathogenesis are crucial aspects to treatment, for improving patient, and renal outcomes.

The Prevalence and Characteristics of Circulating IgA Anti-Glomerular Basement Membrane Autoantibodies in Anti-Glomerular Basement Membrane Disease

In some cases, immunoglobulin (IgA)-mediated antiglomerular basement membrane (anti-GBM) disease has been reported. Whether circulating IgA anti-GBM antibodies affect the clinico-pathologic characteristics and outcome of typical anti-GBM disease deserves further study. Circulating IgA anti-α3(IV)NC1 antibodies were examined by enzyme-linked immunosorbent assay (ELISA) using recombinant human α3(IV)NC1 as solid phase antigens in 107 patients with anti-GBM disease and 115 controls. Clinical, pathological, and follow-up data of patients were retrospectively analyzed. Circulating IgA anti-α3(IV)NC1 antibodies were found in 18.7% (20/107) of patients with anti-GBM disease but were not detected in healthy controls or in patients with other glomerular diseases. The positivity of circulating IgA anti-α3(IV)NC1 antibodies was not associated with whether the patient was with combined IgA nephropathy or other glomerulonephritis. Kidney immunofluorescence showed no statistical difference in IgA deposition between patients with circulating IgA anti-α3(IV)NC1 antibodies and patients without (30.0% vs. 40.4%, P= 0.725). The titers of circulating immunoglobulin G (IgG) anti-α3(IV)NC1 antibodies in patients with circulating IgA anti-α3(IV)NC1 antibodies were significantly higher than those without (200 [183.3, 200] vs. 161 [85.5, 200] U/ml, P= 0.005). There were no significant differences in kidney outcome and mortality between the 2 groups. Circulating IgA anti-α3(IV)NC1 antibodies occurred in 18.7% (20/107) of patients with anti-GBM in our center and were specific to anti-GBM disease. Patients with circulating IgA anti-α3(IV)NC1 antibodies showed a higher levels of serum IgG anti-α3(IV)NC1 antibodies than those without.

Autoantibodies against laminin-521 are pathogenic in anti-glomerular basement membrane disease

Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disorder characterized by autoantibodies against GBM components. Evidence from human inherited kidney diseases and animal models suggests that the α, β, and γ chains of laminin-521 are all essential for maintaining the glomerular filtration barrier. We previously demonstrated that laminin-521 is a novel autoantigen within the GBM and that autoantibodies to laminin-521 are present in about one-third of patients. In the present study, we investigated the pathogenicity of autoantibodies against laminin-521 with clinical and animal studies. Herein, a rare case of anti-GBM disease was reported with circulating autoantibodies binding to laminin-521 but not to the NC1 domains of α1-α5(IV) collagen. Immunoblot identified circulating IgG from this patient bound laminin α5 and γ1 chains. A decrease in antibody levels was associated with improved clinical presentation after plasmapheresis and immunosuppressive treatments. Furthermore, immunization with laminin-521 in female Wistar-Kyoto rats induced crescentic glomerulonephritis with linear IgG deposits along the GBM, complement activation along with infiltration of T cells and macrophages. Lung hemorrhage occurred in 75.0% of the rats and was identified by the presence of erythrocyte infiltrates and hemosiderin-laden macrophages in the lung tissue. Sera and kidney-eluted antibodies from rats immunized with laminin-521 demonstrated specific IgG binding to laminin-521 but not to human α3(IV)NC1, while the opposite was observed in human α3(IV)NC1-immunized rats. Thus, our patient data and animal studies imply a possible independent pathogenic role of autoantibodies against laminin-521 in the development of anti-GBM disease.

Thirteen Cases of Pulmonary Pneumocystis in HIV-Negative Patients.

We report 13 cases of pulmonary pneumocystis (PCP) in human immunodeficiency virus (HIV)-uninfected patients. Of eight males and five females, with a mean age of 55 years, one had breast neoplasia, two had common variable immunodeficiency (CVID), one had an autoimmune disease "Goodpasture's syndrome", and one had idiopathic fibrosis (nonspecific interstitial pneumonia/fibrosis(NIP)) undergoing prolonged corticosteroid therapy for two years, with no known immunosuppression in the remaining cases. The clinical picture was characterized by constant dyspnea and severe hypoxia in 11 cases. Lymphopenia was present in nine caseswith an average rate of 920.76 elements/mm3. The diagnosis was confirmed by isolation of Pneumocystis jirovecii(PCJ) from induced sputum, except in two cases where analysis of bronchoalveolar lavage (BAL) fluid was required. With trimethoprim/sulfamethoxazole (TMP/SMX) and corticosteroid therapy, the course was favorable in all cases. Prophylactic treatment was indicated in three cases.

Impact of concurrent glomerulonephritis on outcomes of diffuse alveolar haemorrhage in antineutrophil cytoplasmic antibody-associated vasculitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) commonly presents with diffuse alveolar haemorrhage (DAH) and/or glomerulonephritis. Patients who present with DAH but without kidney involvement have been understudied. Patients with DAH diagnosed by bronchoscopy and attributed to AAV over 8.5 years were retrospectively identified through electronic medical records and bronchoscopy reporting software. Patients with end-stage kidney disease (ESKD) or prior kidney transplant were excluded. Characteristics, treatments, and outcomes were abstracted. 30 patients were identified with DAH secondary to AAV. Five with ESKD or prior kidney transplant, and one with concomitant anti-glomerular basement membrane disease, were excluded, leaving 24 patients for analysis. At the time of qualifying bronchoscopy, six patients had no apparent kidney involvement by AAV, while eight of 18 with kidney involvement required dialysis. Of the eight patients dialysed during the initial hospitalisation, four were declared to have ESKD and three died in the subsequent year (one of whom did both). None of the 16 patients without initial dialysis requirement developed kidney involvement requiring dialysis in the subsequent year, though three of the six without initial evidence of kidney involvement by AAV ultimately developed it. No patient without initial kidney involvement died during follow-up. In our cohort, patients with DAH due to AAV without initial kidney involvement did not develop kidney involvement requiring dialysis or die during the follow-up period, though half of patients without initial evidence of kidney involvement subsequently developed it. Larger studies are warranted to better characterise this population and guide medical management.