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  1. Article ; Online: Treatment of Fabry Nephropathy: A Literature Review.

    Shimohata, Homare / Yamashita, Marina / Yamada, Kota / Hirayama, Kouichi / Kobayashi, Masaki

    Medicina (Kaunas, Lithuania)

    2023  Volume 59, Issue 8

    Abstract: Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry ... ...

    Abstract Fabry disease is an X-linked inherited lysosomal storage disorder with a deficiency of α-galactosidase A activity, which results in the intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in various organs. Fabry nephropathy is one of the major complications of Fabry disease, and kidney damage is often related to cardiovascular disease and mortality. The treatment of Fabry nephropathy thus helps prolong life expectancy. Two treatment options for Fabry nephropathy and cardiopathy are now commercially available: enzyme replacement therapy (agalsidase α agalsidase β, and a biosimilar of agalsidase β) and pharmacological chaperone therapy (migalastat). In this review, we summarize the efficacy of these treatment options for Fabry nephropathy with respect to renal function, proteinuria, and renal pathological findings. We also describe the importance of adjunctive therapy for Fabry nephropathy.
    MeSH term(s) Humans ; Fabry Disease/complications ; Fabry Disease/drug therapy ; Kidney Diseases ; Dental Care ; Kidney ; Cardiovascular Diseases
    Language English
    Publishing date 2023-08-17
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2188113-3
    ISSN 1648-9144 ; 1010-660X
    ISSN (online) 1648-9144
    ISSN 1010-660X
    DOI 10.3390/medicina59081478
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Staphylococcus aureus

    Takayasu, Mamiko / Hirayama, Kouichi / Shimohata, Homare / Kobayashi, Masaki / Koyama, Akio

    International journal of molecular sciences

    2022  Volume 23, Issue 13

    Abstract: Since 1995, when we reported the case of a patient with glomerulonephritis with IgA deposition that occurred after a methicillin- ... ...

    Abstract Since 1995, when we reported the case of a patient with glomerulonephritis with IgA deposition that occurred after a methicillin-resistant
    MeSH term(s) Glomerulonephritis/pathology ; Glomerulonephritis, IGA/complications ; Glomerulonephritis, IGA/pathology ; Humans ; Immunoglobulin A ; Methicillin-Resistant Staphylococcus aureus ; Staphylococcal Infections ; Staphylococcus aureus
    Chemical Substances Immunoglobulin A
    Language English
    Publishing date 2022-07-05
    Publishing country Switzerland
    Document type Case Reports ; Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms23137482
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Staphylococcus aureus Infection-Related Glomerulonephritis with Dominant IgA Deposition

    Mamiko Takayasu / Kouichi Hirayama / Homare Shimohata / Masaki Kobayashi / Akio Koyama

    International Journal of Molecular Sciences, Vol 23, Iss 7482, p

    2022  Volume 7482

    Abstract: Since 1995, when we reported the case of a patient with glomerulonephritis with IgA deposition that occurred after a methicillin-resistant Staphylococcus aureus (MRSA) infection, many reports of MRSA infection-associated glomerulonephritis have ... ...

    Abstract Since 1995, when we reported the case of a patient with glomerulonephritis with IgA deposition that occurred after a methicillin-resistant Staphylococcus aureus (MRSA) infection, many reports of MRSA infection-associated glomerulonephritis have accumulated. This disease is being systematized as Staphylococcus infection-associated glomerulonephritis (SAGN) in light of the apparent cause of infection, and as immunoglobulin A-dominant deposition infection-related glomerulonephritis (IgA-IRGN) in light of its histopathology. This glomerulonephritis usually presents as rapidly progressive glomerulonephritis or acute kidney injury with various degrees of proteinuria and microscopic hematuria along with an ongoing infection. Its renal pathology has shown several types of mesangial and/or endocapillary proliferative glomerulonephritis with various degrees of crescent formation and tubulointerstitial nephritis. IgA, IgG, and C 3 staining in the mesangium and along the glomerular capillary walls have been observed on immunofluorescence examinations. A marked activation of T cells, an increase in specific variable regions of the T-cell receptor β-chain-positive cells, hypercytokinemia, and increased polyclonal immune complexes have also been observed in this glomerulonephritis. In the development of this disease, staphylococcal enterotoxin may be involved as a superantigen, but further investigations are needed to clarify the mechanisms underlying this disease. Here, we review 336 cases of IgA-IRGN and 218 cases of SAGN.
    Keywords Staphylococcus aureus ; rapidly progressive glomerulonephritis ; IgA-dominant glomerulonephritis ; Staphylococcus infection-associated glomerulonephritis ; bacterial superantigen ; T-cell receptor ; Biology (General) ; QH301-705.5 ; Chemistry ; QD1-999
    Subject code 616
    Language English
    Publishing date 2022-07-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: NT-pro BNP level at dialysis initiation is a useful biomarker for predicting hospitalization for ischemic heart disease.

    Shimohata, Homare / Usui, Joichi / Tawara-Iida, Takashi / Ebihara, Itaru / Ishizu, Takashi / Maeda, Yoshitaka / Kobayashi, Hiroaki / Numajiri, Daichi / Kaneshige, Ayaka / Sega, Masatoshi / Yamashita, Marina / Ohgi, Kentaro / Maruyama, Hiroshi / Takayasu, Mamiko / Hirayama, Kouichi / Kobayashi, Masaki / Yamagata, Kunihiro

    Clinical and experimental nephrology

    2024  Volume 28, Issue 5, Page(s) 457–464

    Abstract: Background: Patients with end-stage kidney disease (ESKD) are at high risk of cardiovascular disease including stroke, heart failure, and ischemic heart disease (IHD). To prevent the occurrence and progression of CVD, a reliable prognostic cardiac ... ...

    Abstract Background: Patients with end-stage kidney disease (ESKD) are at high risk of cardiovascular disease including stroke, heart failure, and ischemic heart disease (IHD). To prevent the occurrence and progression of CVD, a reliable prognostic cardiac biomarker is essential. We investigated the prognostic value of NT-proBNP for each incident type of CVD.
    Methods: Male patients from the Ibaraki Dialysis Initiation Cohort (iDIC) study with preserved serum samples from dialysis initiation day (n = 212) were analyzed. Patients were classified into four groups according to quartiles of baseline NT-pro BNP levels. The relationship between NT-proBNP levels at the initiation of dialysis and the subsequent incidence of hospitalization events due to IHD, heart failure, and stroke was analyzed.
    Results: The incidence rate for hospitalization due to IHD was significantly higher in the highest NT-proBNP category (Log rank p = 0.008); those of stroke and heart failure showed no significant differences among quartiles. Cox proportional hazards regression analysis revealed that serum NT-proBNT was the only prognostic factor for hospitalization for IHD after adjustment by major known IHD risk factors. (HR, 1.008; 95% confidence interval, 1.002-1.014; p = 0.01) The ROC curve analysis for the incidence of hospitalization due to IHD showed that NT-proBNP had an area under the curve (AUC) of 0.759 (95% CI 0.622-0.897; p = 0.004) at a cut-off value of 956.6 pg/mL.
    Conclusion: NT-proBNP measurement at the initiation of dialysis therapy is useful to predict later hospitalization for IHD.
    Trial registration: UMIN000010806.
    MeSH term(s) Humans ; Male ; Natriuretic Peptide, Brain/blood ; Biomarkers/blood ; Peptide Fragments/blood ; Myocardial Ischemia/blood ; Myocardial Ischemia/epidemiology ; Myocardial Ischemia/diagnosis ; Renal Dialysis ; Middle Aged ; Aged ; Hospitalization ; Kidney Failure, Chronic/therapy ; Kidney Failure, Chronic/blood ; Kidney Failure, Chronic/complications ; Heart Failure/blood ; Heart Failure/therapy ; Heart Failure/epidemiology ; Prognosis ; Incidence ; Stroke/blood ; Stroke/epidemiology ; Predictive Value of Tests ; ROC Curve ; Proportional Hazards Models ; Japan/epidemiology
    Chemical Substances Natriuretic Peptide, Brain (114471-18-0) ; pro-brain natriuretic peptide (1-76) ; Biomarkers ; Peptide Fragments
    Language English
    Publishing date 2024-01-19
    Publishing country Japan
    Document type Journal Article
    ZDB-ID 1338768-6
    ISSN 1437-7799 ; 1342-1751
    ISSN (online) 1437-7799
    ISSN 1342-1751
    DOI 10.1007/s10157-023-02442-x
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Clinical course and pathological findings of two late-onset Fabry hemizygous patients including mulberry cell counts after enzyme replacement therapy.

    Shimohata, Homare / Yamashita, Marina / Ohgi, Kentaro / Maruyama, Hiroshi / Takayasu, Mamiko / Hirayama, Kouichi / Kobayashi, Masaki

    CEN case reports

    2020  Volume 9, Issue 3, Page(s) 237–242

    Abstract: Fabry disease is an X-linked inherited lysosomal storage disorder caused by a deficiency of α-galactosidase A activity, resulting in the intracellular accumulation of globotriaosylceramide and related glycosphingolipids. The phenotypes of Fabry disease ... ...

    Abstract Fabry disease is an X-linked inherited lysosomal storage disorder caused by a deficiency of α-galactosidase A activity, resulting in the intracellular accumulation of globotriaosylceramide and related glycosphingolipids. The phenotypes of Fabry disease in both males and females are grouped into two categories: the classical type and the late-onset type. The classical type shows general symptoms including angiokeratoma(s), acroparesthesia, hypohidrosis, corneal opacity, and gastrointestinal symptoms from an early age. The late-onset type shows cardiac or renal (or both) symptoms from a late age. We present herein the clinical course and pathological findings of two late-onset hemizygous Fabry patients after the initiation of enzyme replacement therapy (ERT), along with their mulberry cell counts during treatment. One patient's case was a renal-variant type without general symptoms; he showed stable renal function and mild proteinuria but little histological improvement with no change in the mulberry cell count during ERT. The other patient had a cardiac-variant type with renal pathological abnormality. He achieved a mild improvement of renal pathological findings, and his mulberry cell count gradually decreased during the treatment. These findings indicate that monitoring the mulberry cell count might help assess the efficacy of ERT, as a renal pathology tool.
    MeSH term(s) Adult ; Asian People/ethnology ; Biopsy/methods ; Cardiomyopathies/diagnosis ; Cardiomyopathies/etiology ; Cell Count/methods ; Enzyme Replacement Therapy/methods ; Fabry Disease/diagnosis ; Fabry Disease/genetics ; Fabry Disease/pathology ; Fabry Disease/therapy ; Glycosphingolipids/analysis ; Humans ; Kidney/abnormalities ; Kidney/pathology ; Kidney Diseases/etiology ; Kidney Diseases/pathology ; Kidney Diseases/physiopathology ; Kidney Diseases/therapy ; Late Onset Disorders/diagnosis ; Late Onset Disorders/genetics ; Late Onset Disorders/pathology ; Male ; Microscopy, Electron/methods ; Middle Aged ; Morus ; Phenotype ; Podocytes/pathology ; Podocytes/ultrastructure ; Proteinuria/diagnosis ; Proteinuria/etiology ; Treatment Outcome
    Chemical Substances Glycosphingolipids
    Language English
    Publishing date 2020-03-18
    Publishing country Japan
    Document type Journal Article ; Case Reports
    ZDB-ID 2660492-9
    ISSN 2192-4449 ; 2192-4449
    ISSN (online) 2192-4449
    ISSN 2192-4449
    DOI 10.1007/s13730-020-00463-z
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: LMX1B-associated nephropathy that showed myelin figures on electron microscopy.

    Shimohata, Homare / Miyake, Yusuke / Yoshida, Yu / Usui, Joichi / Mori, Takayasu / Sohara, Eisei / Uchida, Shinichi / Hirayama, Kouichi / Kobayashi, Masaki

    CEN case reports

    2021  Volume 10, Issue 4, Page(s) 588–591

    Abstract: The mutation of LIM homeodomain transcription factor LMX1B gene leads to nail-patella syndrome (NPS), which is characterized by dysplastic nails, hypoplastic patellae, iliac horns and nephropathy. The characteristic renal histological finding of NPS ... ...

    Abstract The mutation of LIM homeodomain transcription factor LMX1B gene leads to nail-patella syndrome (NPS), which is characterized by dysplastic nails, hypoplastic patellae, iliac horns and nephropathy. The characteristic renal histological finding of NPS nephropathy is irregular thickening of the glomerular basement membrane with patchy lucent areas, including deposits of bundles of type III collagen fibrils revealed by electron microscopy (EM). Fabry disease is a lysosomal storage disorder caused by a deficiency of α-galactosidase A activity, and the characteristic EM finding is a lamellated membrane structure (myelin figures). We present the case of a male with LMX1B-associated nephropathy (LAN) who showed focal segmental glomerulosclerosis (FSGS) on light microscopy, and myelin figures and slight deposits of collagen fibrils on EM, without findings of glomerular basement membrane abnormality suggestive for NPS. A 21-year-old Japanese-Brazilian man was admitted to hospital for an investigation of the cause of proteinuria and decreased renal function. A renal biopsy was performed to investigate the cause of renal damage. Fabry disease was initially considered, based on the presence of myelin figures on EM, but since he had normal α-galactosidase A activity, this initial diagnosis was denied, and the patient was subsequently diagnosed with FSGS. At 22 years after that renal biopsy, the patient was incidentally diagnosed with LAN when NM_002316:3c.746G > A:p.(Arg249Gln) LMX1B variant was identified in his older brother by a pre-transplantation examination, and the same mutation was confirmed in the patient. Myelin figures revealed by EM might become one of the clues for the diagnosis of LAN.
    MeSH term(s) Glomerulosclerosis, Focal Segmental/genetics ; Glomerulosclerosis, Focal Segmental/metabolism ; Glomerulosclerosis, Focal Segmental/pathology ; Humans ; LIM-Homeodomain Proteins/genetics ; Male ; Myelin Sheath/metabolism ; Podocytes/ultrastructure ; Transcription Factors/genetics ; Young Adult
    Chemical Substances LIM homeobox transcription factor 1 beta ; LIM-Homeodomain Proteins ; Transcription Factors
    Language English
    Publishing date 2021-06-02
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 2660492-9
    ISSN 2192-4449 ; 2192-4449
    ISSN (online) 2192-4449
    ISSN 2192-4449
    DOI 10.1007/s13730-021-00612-y
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: The effect of sodium-glucose cotransporter 2 inhibitor (tofogliflozin) on renal tubular damage in diabetic patients without albuminuria.

    Shimohata, Homare / Iwaki, Yoshiki / Yamashita, Marina / Ohgi, Kentaro / Maruyama, Hiroshi / Takayasu, Mamiko / Hirayama, Kouichi / Kobayashi, Masaki

    International urology and nephrology

    2021  Volume 54, Issue 8, Page(s) 1907–1914

    Abstract: Purpose: The sodium-glucose cotransporter 2 (SGLT2) inhibitors comprise a new class of glucose-lowering drugs for individuals with diabetes. Large-scale clinical trials indicated that SGLT2 inhibitors have both a cardiovascular-protective and renal- ... ...

    Abstract Purpose: The sodium-glucose cotransporter 2 (SGLT2) inhibitors comprise a new class of glucose-lowering drugs for individuals with diabetes. Large-scale clinical trials indicated that SGLT2 inhibitors have both a cardiovascular-protective and renal-protective effects. A reduction in glomerular hyperfiltration and a decrease in albuminuria are suspected as the main causes of SGLT2 inhibitors' renoprotective effect. The effects of SGLT2 inhibitors on tubular damage in non-albuminuric diabetic patients are unclear.
    Methods: The SGLT2 inhibitor tofogliflozin (20 mg, 1 × /day) was orally administered to 14 non-albuminuric diabetic patients. Serum and urine samples were collected at baseline (before) and after the start of tofogliflozin treatment. Hemoglobin A1c, hemoglobin, estimated glomerular filtration rate (eGFR), body weight, and blood pressure (BP) were analyzed as clinical parameters at baseline and 1, 3, and 6 months later. Urinary neutrophil gelatinase-associated lipocalin (NGAL) and N-acetyl-β-D-glucosaminidase were measured as tubular damage markers and the urinary 8-hidroxydeoxyguanosine (8-OHdG) values were measured as an oxidative stress marker at baseline and at 1 and 3 months.
    Results: Compared to baseline, the patients' HbA1c values and body weights were significantly decreased post-tofogliflozin administration, and their eGFR values were decreased at 3 months but recovered at 6 months; the hemoglobin concentrations were significantly increased at 3 and 6 months and the urinary NGAL level tended to be decreased at 3 months. No significant changes in blood urea nitrogen, BP, NAG, urine sodium concentration, or urinary 8-OHdG values occurred. The effect of this SGLT2 inhibitor was not influenced by the use of an angiotensin receptor blocker or dipeptidyl-peptidase 4 inhibitor.
    Conclusion: For individuals with non-albuminuric diabetes, tofogliflozin has a good glucose-lowering effect and might have a tubular-protective effect.
    MeSH term(s) Albuminuria/drug therapy ; Albuminuria/etiology ; Benzhydryl Compounds ; Biomarkers ; Blood Glucose ; Diabetes Mellitus, Type 2/complications ; Diabetes Mellitus, Type 2/drug therapy ; Glucosides ; Humans ; Lipocalin-2 ; Sodium ; Sodium-Glucose Transporter 2/therapeutic use ; Sodium-Glucose Transporter 2 Inhibitors/pharmacology ; Sodium-Glucose Transporter 2 Inhibitors/therapeutic use
    Chemical Substances Benzhydryl Compounds ; Biomarkers ; Blood Glucose ; Glucosides ; Lipocalin-2 ; Sodium-Glucose Transporter 2 ; Sodium-Glucose Transporter 2 Inhibitors ; Sodium (9NEZ333N27) ; 6-((4-ethylphenyl)methyl)-3',4',5',6'-tetrahydro-6'-(hydroxymethyl)spiro(isobenzofuran-1(3H),2'-(2H)pyran)-3',4',5'-triol (P8DD8KX4O4)
    Language English
    Publishing date 2021-11-29
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 204048-7
    ISSN 1573-2584 ; 0301-1623 ; 0042-1162
    ISSN (online) 1573-2584
    ISSN 0301-1623 ; 0042-1162
    DOI 10.1007/s11255-021-03064-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Serum 20S proteasome levels are associated with disease activity in MPO-ANCA-associated microscopic polyangiitis.

    Maruyama, Hiroshi / Hirayama, Kouichi / Yamashita, Marina / Ohgi, Kentaro / Tsujimoto, Ryuji / Takayasu, Mamiko / Shimohata, Homare / Kobayashi, Masaki

    BMC rheumatology

    2020  Volume 4, Page(s) 36

    Abstract: Background: Proteasomes are found in both the cell nucleus and cytoplasm and play a major role in the ubiquitin-dependent and -independent non-lysosomal pathways of intracellular protein degradation. Proteasomes are also involved in the turnover of ... ...

    Abstract Background: Proteasomes are found in both the cell nucleus and cytoplasm and play a major role in the ubiquitin-dependent and -independent non-lysosomal pathways of intracellular protein degradation. Proteasomes are also involved in the turnover of various regulatory proteins, antigen processing, cell differentiation, and apoptosis. To determine the diagnostic value of serum proteasome in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), we investigated patients with AAV at various stages of the disease.
    Methods: Serum 20S-proteasome was measured by ELISA in 44 patients with MPO-ANCA-associated microscopic polyangiitis (MPA) and renal involvement. Thirty of the patients provided serum samples before the initial treatment, and 30 provided samples during remission; 16 provided samples at both time points.
    Results: The mean serum 20S-proteasome level was significantly higher in the active-vasculitis patients (3414.6 ± 2738.9 ng/mL;
    Conclusion: The serum level of 20S-proteasome may be a useful marker for disease activity in AAV.
    Language English
    Publishing date 2020-08-25
    Publishing country England
    Document type Journal Article
    ZDB-ID 2918121-5
    ISSN 2520-1026 ; 2520-1026
    ISSN (online) 2520-1026
    ISSN 2520-1026
    DOI 10.1186/s41927-020-00137-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: An Adult Patient with Alagille Syndrome Showing Mainly Renal Failure and Vascular Abnormality without Liver Manifestation.

    Shimohata, Homare / Imagawa, Kazuo / Yamashita, Marina / Ohgi, Kentaro / Maruyama, Hiroshi / Takayasu, Mamiko / Hirayama, Kouichi / Kobayashi, Masaki

    Internal medicine (Tokyo, Japan)

    2020  Volume 59, Issue 22, Page(s) 2907–2910

    Abstract: Alagille syndrome is an inherited multisystemic disorder. We herein report an atypical case of a Japanese adult patient with Alagille syndrome. He had been diagnosed with Alagille syndrome as an infant based on a liver biopsy. At 27 years of age, he ... ...

    Abstract Alagille syndrome is an inherited multisystemic disorder. We herein report an atypical case of a Japanese adult patient with Alagille syndrome. He had been diagnosed with Alagille syndrome as an infant based on a liver biopsy. At 27 years of age, he needed to start hemodialysis therapy, but an arteriovenous fistula was not created because his peripheral blood vessels were too narrow. He also had a recurrent brain infarction due to cerebral vascular stenosis. Alagille syndrome is generally recognized as a pediatric hepatic disease, but general physicians should be aware of its potential existence with renal involvement and vascular abnormalities.
    MeSH term(s) Adult ; Alagille Syndrome/complications ; Alagille Syndrome/diagnosis ; Biopsy ; Child ; Humans ; Infant ; Liver Diseases ; Male ; Renal Insufficiency
    Language English
    Publishing date 2020-07-28
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 32371-8
    ISSN 1349-7235 ; 0021-5120 ; 0918-2918
    ISSN (online) 1349-7235
    ISSN 0021-5120 ; 0918-2918
    DOI 10.2169/internalmedicine.4780-20
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Microscopic polyangiitis associated with thymic tumor: a case report and review of the literature.

    Miyamoto, Yasunori / Hirayama, Kouichi / Maruyama, Hiroshi / Ohgi, Kentaro / Takayasu, Mamiko / Shimohata, Homare / Kobayashi, Masaki

    BMC nephrology

    2019  Volume 20, Issue 1, Page(s) 123

    Abstract: Background: Thymic hyperplasia and thymic epithelial tumor (thymoma) have been associated with a variety of autoimmune diseases. Renal involvement has been reported in patients with thymoma. Minimal change disease and membranous nephropathy are ... ...

    Abstract Background: Thymic hyperplasia and thymic epithelial tumor (thymoma) have been associated with a variety of autoimmune diseases. Renal involvement has been reported in patients with thymoma. Minimal change disease and membranous nephropathy are frequently observed in glomerular lesions of thymoma patients, but ANCA-associated renal vasculitis is rare. We present a case of thymoma-associated microscopic polyangiitis with positivity for three ANCAs: MPO-ANCA, PR3-ANCA and azurocidin-ANCA.
    Case presentation: An 89-year-old Japanese woman was admitted to our hospital following an episode of general fatigue, nausea, muscle weakness of the lower limbs, and ophthalmoplegia. On urinalysis, proteinuria, hematuria, and cellular casts were observed. Elevated levels of serum creatinine and C-reactive protein were also demonstrated, and MPO-, PR3- and azurocidin-ANCA were detected on serological examination. Renal biopsy showed pauci-immune crescentic glomerulonephritis. We therefore diagnosed rapidly progressive glomerulonephritis due to microscopic polyangiitis. Acetylcholine-receptor antibody was also detected. Chest computed tomography and MRI revealed a lobulated tumor in the anterior mediastinum. We thus also diagnosed myasthenia gravis with thymoma.
    Conclusion: Considering the patient's triple-ANCA positivity, thymic diseases may be associated with the pathogenesis of ANCA-associated vasculitis due to central T-cell tolerance. A further accumulation of cases is needed, because thymectomy does not always induce the remission of thymoma-associated autoimmune diseases.
    MeSH term(s) Aged, 80 and over ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/blood ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis ; Antibodies, Antineutrophil Cytoplasmic/blood ; Antibodies, Antineutrophil Cytoplasmic/classification ; Biopsy/methods ; Diagnosis, Differential ; Disease Progression ; Female ; Humans ; Kidney Glomerulus/pathology ; Magnetic Resonance Imaging/methods ; Mediastinum/diagnostic imaging ; Microscopic Polyangiitis/complications ; Microscopic Polyangiitis/immunology ; Microscopic Polyangiitis/pathology ; Microscopic Polyangiitis/urine ; Patient Care Management ; Thymoma/complications ; Thymoma/diagnosis ; Thymoma/immunology ; Thymoma/pathology ; Thymus Neoplasms/complications ; Thymus Neoplasms/diagnosis ; Thymus Neoplasms/immunology ; Thymus Neoplasms/pathology ; Tomography, X-Ray Computed/methods ; Urinalysis/methods
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic
    Language English
    Publishing date 2019-04-08
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 2041348-8
    ISSN 1471-2369 ; 1471-2369
    ISSN (online) 1471-2369
    ISSN 1471-2369
    DOI 10.1186/s12882-019-1319-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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