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[PMID]: 29367532
[Au] Autor:Michitsuji T; Horai Y; Sako A; Asano T; Iwanaga N; Izumi Y; Kawakami A
[Ad] Address:Department of General and Internal Medicine, National Hospital Organization Nagasaki Medical Center.
[Ti] Title:[A case of mixed connective tissue disease positive for proteinase 3 antineutrophil cytoplasmic antibody in a patient with slowly progressive type 1 diabetes mellitus and chronic thyroiditis].
[So] Source:Nihon Rinsho Meneki Gakkai Kaishi;40(6):467-470, 2017.
[Is] ISSN:1349-7413
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:  A female in her sixties with slowly progressive type 1 diabetes mellitus (SPT1DM) and chronic thyroiditis was referred to our rheumatology department with swelling in her fingers. A prominent atherosclerotic lesion was revealed upon brain magnetic resonance imaging, and she was found to have mixed connective tissue disease (MCTD) positive for proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA). This rare case of MCTD accompanying SPT1DM and PR3-ANCA suggested that a synergy between MCTD and PR3-ANCA triggers atherosclerosis.
[Mh] MeSH terms primary: Antibodies, Antineutrophil Cytoplasmic
Atherosclerosis/etiology
Diabetes Mellitus, Type 1/complications
Hashimoto Disease/complications
Mixed Connective Tissue Disease/diagnosis
Mixed Connective Tissue Disease/immunology
Myeloblastin/immunology
Thyroiditis/complications
[Mh] MeSH terms secundary: Aged
Atherosclerosis/diagnostic imaging
Atherosclerosis/immunology
Brain/diagnostic imaging
Disease Progression
Female
Humans
Magnetic Resonance Imaging
Middle Aged
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); EC 3.4.21.76 (Myeloblastin)
[Em] Entry month:1802
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[Js] Journal subset:IM
[Da] Date of entry for processing:180126
[St] Status:MEDLINE
[do] DOI:10.2177/jsci.40.467

  2 / 1084 MEDLINE  
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[PMID]: 29238021
[Au] Autor:Kiboshi T; Isoda K; Furukawa K; Wakahara T; Otani K; Ueda K; Konma J; Teramura K; Ueno N; Fujiwara H; Shoda T
[Ad] Address:Department of Rheumatology, Yodogawa Christian Hospital.
[Ti] Title:[Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature].
[So] Source:Nihon Rinsho Meneki Gakkai Kaishi;40(5):382-386, 2017.
[Is] ISSN:1349-7413
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:  A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes. Gastrointestinal perforation in GPA is a rare complication, and we examined the clinical features, treatment contents, and prognosis of GPA with gastrointestinal perforation from this case and previous reports. Lung involvements were complicated in all reported cases. Gastrointestinal perforations in GPA were frequent in the small intestine, occurred just before and immediately after the start of treatment, and were severe involvement with poor prognosis because of the high mortality rate (46.7%). The frequency of ear, nose and upper respiratory tract lesions in the surviving group was significantly higher than in the dead group (survival 87.5%, death 28.3%, P = 0.041). IVCY were more frequently used in the surviving group (62.5%) than the death group (16.7%), but it was not significantly. GPA complicated with gastrointestinal perforation is a severe condition with poor prognosis, but there is a possibility to improve prognosis by early diagnosis and early initiation of strong treatment.
[Mh] MeSH terms primary: Granulomatosis with Polyangiitis/complications
Granulomatosis with Polyangiitis/therapy
Ileum
Intestinal Perforation/etiology
Plasma Exchange
[Mh] MeSH terms secundary: Antibodies, Antineutrophil Cytoplasmic/blood
Azathioprine/administration & dosage
Biomarkers/blood
Cyclophosphamide/administration & dosage
Early Diagnosis
Granulomatosis with Polyangiitis/diagnosis
Humans
Intestinal Perforation/surgery
Male
Methylprednisolone/administration & dosage
Middle Aged
Myeloblastin/immunology
Prognosis
Pulse Therapy, Drug
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); 0 (Biomarkers); 8N3DW7272P (Cyclophosphamide); EC 3.4.21.76 (Myeloblastin); MRK240IY2L (Azathioprine); X4W7ZR7023 (Methylprednisolone)
[Em] Entry month:1801
[Cu] Class update date: 180112
[Lr] Last revision date:180112
[Js] Journal subset:IM
[Da] Date of entry for processing:171215
[St] Status:MEDLINE
[do] DOI:10.2177/jsci.40.382

  3 / 1084 MEDLINE  
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[PMID]: 28745694
[Au] Autor:Androsova TV; Kozlovskaya LV; Taranova MV; Strizhakov LA; Gulyaev SV; Russkikh AV
[Ad] Address:I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia, Moscow, Russia.
[Ti] Title:Trudnosti differentsial'noi diagnostiki porazheniia pochek u bol'nogo infektsionnym éndokarditom, assotsiirovannym s antineitrofil'nymi tsitoplazmaticheskimi antitelami. [Difficulties in the differential diagnosis of kidney injury in a patient with infective endocarditis associated with antineutrophil cytoplasmic antibodies].
[So] Source:Ter Arkh;89(6):84-88, 2017.
[Is] ISSN:0040-3660
[Cp] Country of publication:Russia (Federation)
[La] Language:rus
[Ab] Abstract:Infective endocarditis (IE) may be accompanied by the production of a broad spectrum of autoantibodies, including antineutrophil cytoplasmic antibodies (ANCA). ANCA detection creates difficulties in the differential diagnosis of IE, especially in relation to kidney injury, the determination of the mechanism of which is important for choosing a treatment policy and estimating a prognosis. The paper describes a clinical case of a 57-year-old man who was found to have higher proteinase-3 (PR-3) ANCA titers along with the symptoms of anemia, purpura, and kidney injury during his hospitalization; echocardiography revealed vegetation on the aortic valve. IE was diagnosed; 2-week antibiotic therapy was ineffective; there was progressive aortic insufficiency necessitating aortic valve replacement. In the postoperative period, there was progression of renal failure and higher PR-3 ANCA titers, which made it possible to regard kidney injury as a manifestation of ANCA-associated glomerulonephritis. Intensive immunosuppressive therapy with intravenous and oral prednisolone was initiated, which showed positive effects in reducing proteinuria, erythrocyturia, serum creatinine levels, and simultaneously PR-3 ANCA titers. The paper gives the data available in the literature on the frequency of an association of IE with ANCA, the clinical features, diagnostic criteria, and treatment approaches. It discusses the mechanisms of ANCA formation in patients with IE.
[Mh] MeSH terms primary: Antibodies, Antineutrophil Cytoplasmic/blood
Endocarditis/blood
Glomerulonephritis/diagnosis
Myeloblastin/blood
[Mh] MeSH terms secundary: Endocarditis/complications
Humans
Male
Middle Aged
[Pt] Publication type:CASE REPORTS
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); EC 3.4.21.76 (Myeloblastin)
[Em] Entry month:1711
[Cu] Class update date: 171129
[Lr] Last revision date:171129
[Js] Journal subset:IM
[Da] Date of entry for processing:170727
[St] Status:MEDLINE
[do] DOI:10.17116/terarkh201789684-88

  4 / 1084 MEDLINE  
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[PMID]: 28981267
[Au] Autor:Stolle T; Grondinger F; Dunkel A; Meng C; Médard G; Kuster B; Hofmann T
[Ad] Address:Chair of Food Chemistry and Molecular Sensory Science, Technical University of Munich , Lise-Meitner Strasse 34, D-85354 Freising, Germany.
[Ti] Title:Salivary Proteome Patterns Affecting Human Salt Taste Sensitivity.
[So] Source:J Agric Food Chem;65(42):9275-9286, 2017 Oct 25.
[Is] ISSN:1520-5118
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:To investigate the role of perireceptor events in inter-individual variability in salt taste sensitivity, 31 volunteers were monitored in their detection functions for sodium chloride (NaCl) and classified into sensitive (0.6-1.7 mmol/L), medium-sensitive (1.8-6.9 mmol/L), and nonsensitive (7.0-11.2 mmol/L) subjects. Chemosensory intervention of NaCl-sensitive (S ) and nonsensitive (S ) panellists with potassium chloride, ammonium chloride, and sodium gluconate showed the salt taste sensitivity to be specific for NaCl. As no significant differences were found between S and S subjects in salivary sodium and protein content, salivary proteome differences and their stimulus-induced dynamic changes were analyzed by tryptic digestion, iTRAQ labeling, and liquid chromatography-tandem mass spectrometry analysis. Differences in the salivary proteome between S and S subjects were found primarily in resting saliva and were largely independent of the dynamic alterations observed upon salt stimulation. Gene ontology enrichment analysis of key proteins, i.e., immunoglobulin heavy constant y1, myeloblastin, cathepsin G, and kallikrein, revealed significantly increased serine-type endopeptidase activity for the S group, while the S group exhibited augmented cysteine-type endopeptidase inhibitor activity by increased abundances in lipocalin-1 and cystatin-D, -S, and -SN, respectively. As proteases have been suggested to facilitate transepithelial sodium transport by cleaving the y-subunit of the epithelial sodium channel (ENaC) and protease inhibitors have been shown to reduce ENaC-mediated sodium transport, the differentially modulated proteolytic activity patterns observed in vivo for S and S subjects show evidence of them playing a crucial role in affecting human NaCl sensitivity.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1710
[Cu] Class update date: 171025
[Lr] Last revision date:171025
[St] Status:In-Process
[do] DOI:10.1021/acs.jafc.7b03862

  5 / 1084 MEDLINE  
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[PMID]: 28977502
[Au] Autor:de Joode AAE; Sanders JSF; Puéchal X; Guillevin LP; Hiemstra TF; Flossmann O; Rasmussen N; Westman K; Jayne DR; Stegeman CA
[Ad] Address:Department of Internal Medicine and Nephrology, University Medical Center Groningen, Groningen, the Netherlands.
[Ti] Title:Long term azathioprine maintenance therapy in ANCA-associated vasculitis: combined results of long-term follow-up data.
[So] Source:Rheumatology (Oxford);56(11):1894-1901, 2017 Nov 01.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objective: We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up. Methods: Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48.3-68.2 years); granulomatosis with polyangiitis, n = 236; microscopic polyangiitis, n = 132; or renal limited vasculitis, n = 12. Patients were grouped according to the duration of AZA maintenance after remission induction: ⩽18 months, ⩽24 months, ⩽36 months, ⩽48 months or > 48 months. Primary outcome was relapse-free survival at 60 months. Results: During follow-up, 84 first relapses occurred during AZA-maintenance therapy (1 relapse per 117 patient months) and 71 after withdrawal of AZA (1 relapse/113 months). During the first 12 months after withdrawal, 20 relapses occurred (1 relapse/119 months) and 29 relapses >12 months after withdrawal (1 relapse/186 months). Relapse-free survival at 60 months was 65.3% for patients receiving AZA maintenance >18 months after diagnosis vs 55% for those who discontinued maintenance ⩽18 months (P = 0.11). Relapse-free survival was associated with induction therapy (i.v. vs oral) and ANCA specificity (PR3-ANCA vs MPO-ANCA/negative). Conclusion: Post hoc analysis of combined trial data suggest that stopping AZA maintenance therapy does not lead to a significant increase in relapse rate and AZA maintenance for more than 18 months after diagnosis does not significantly influence relapse-free survival. ANCA specificity has more effect on relapse-free survival than duration of maintenance therapy and should be used to tailor therapy individually.
[Mh] MeSH terms primary: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy
Azathioprine/therapeutic use
Immunosuppressive Agents/therapeutic use
[Mh] MeSH terms secundary: Adult
Aged
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology
Antibodies, Antineutrophil Cytoplasmic/immunology
Disease-Free Survival
Female
Follow-Up Studies
Granulomatosis with Polyangiitis/drug therapy
Granulomatosis with Polyangiitis/immunology
Humans
Kidney Diseases/drug therapy
Kidney Diseases/immunology
Maintenance Chemotherapy
Male
Microscopic Polyangiitis/drug therapy
Microscopic Polyangiitis/immunology
Middle Aged
Myeloblastin/immunology
Peroxidase/immunology
Recurrence
Time Factors
Young Adult
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); 0 (Immunosuppressive Agents); EC 1.11.1.7 (Peroxidase); EC 3.4.21.76 (Myeloblastin); MRK240IY2L (Azathioprine)
[Em] Entry month:1711
[Cu] Class update date: 171106
[Lr] Last revision date:171106
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:171005
[St] Status:MEDLINE
[do] DOI:10.1093/rheumatology/kex281

  6 / 1084 MEDLINE  
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[PMID]: 28968886
[Au] Autor:Pearce FA; Craven A; Merkel PA; Luqmani RA; Watts RA
[Ad] Address:Division of Epidemiology and Public Health, University of Nottingham.
[Ti] Title:Global ethnic and geographic differences in the clinical presentations of anti-neutrophil cytoplasm antibody-associated vasculitis.
[So] Source:Rheumatology (Oxford);56(11):1962-1969, 2017 Nov 01.
[Is] ISSN:1462-0332
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objectives: There are few data on clinical profiles of ANCA-associated vasculitis (AAV) in different ethnic populations. The aim of this study was to examine the differences in the ANCA type and clinical features of AAV between populations using the Diagnostic and Classification Criteria in Vasculitis Study (DCVAS) dataset. Methods: The DCVAS is an international, multicentre, observational study recruiting in 133 sites. Eight ethnic categories were analysed: Northern European, Caucasian American, Southern European, Middle Eastern/Turkish, Chinese, Japanese, Indian subcontinent and other. ANCA type was categorized as myeloperoxidase (MPO), PR3 and ANCA negative. Organ system involvement was recorded using a standard dataset. Differences were analysed by chi-squared tests using a Bonferroni correction and logistic regression (adjusting for age and sex). Northern European was the reference population. Results: Data from 1217 patients with AAV were available and the 967 (79.5%) patients recruited by rheumatology departments were analysed to reduce confounding by recruitment specialty. There were differences in ANCA type between ethnic categories (P < 0.001): MPO-ANCA was more common than PR3-ANCA in Japanese, Chinese and Southern Europeans; PR3-ANCA was more common in the other groups. Compared with Northern Europeans, Japanese had a nearly 60-fold increased chance of having MPO-ANCA (vs PR3-ANCA) [odds ratio (OR) 59.2 (95% CI 8.0, 440.7), P < 0.001] and Chinese had a nearly 7-times increased chance [OR 6.8 (95% CI 2.6, 17.8), P < 0.001]. Ophthalmologic and otorhinolaryngologic involvement were less common in Japanese and Chinese populations than Northern Europeans; otherwise, there were few differences in organ involvement between ethnic groups. Conclusion: This study confirms the previously observed differential occurrence of MPO-AAV and PR3-AAV between different ethnic groups.
[Mh] MeSH terms primary: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/physiopathology
Eye Diseases/physiopathology
Kidney Diseases/physiopathology
Otorhinolaryngologic Diseases/physiopathology
Skin Diseases/physiopathology
[Mh] MeSH terms secundary: Aged
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/ethnology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology
Antibodies, Antineutrophil Cytoplasmic/immunology
Arabs
Asian Continental Ancestry Group
China/epidemiology
Europe/epidemiology
European Continental Ancestry Group
Eye Diseases/etiology
Female
Female Urogenital Diseases/etiology
Female Urogenital Diseases/physiopathology
Heart Diseases/etiology
Heart Diseases/physiopathology
Humans
India/epidemiology
Japan/epidemiology
Kidney Diseases/etiology
Male
Male Urogenital Diseases/etiology
Male Urogenital Diseases/physiopathology
Middle Aged
Middle East/epidemiology
Myeloblastin/immunology
Nervous System Diseases/etiology
Nervous System Diseases/physiopathology
Odds Ratio
Otorhinolaryngologic Diseases/etiology
Peroxidase/immunology
Respiratory Tract Diseases/etiology
Respiratory Tract Diseases/physiopathology
Skin Diseases/etiology
Turkey/epidemiology
United States/epidemiology
[Pt] Publication type:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); EC 1.11.1.7 (Peroxidase); EC 3.4.21.76 (Myeloblastin)
[Em] Entry month:1711
[Cu] Class update date: 171106
[Lr] Last revision date:171106
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:171003
[St] Status:MEDLINE
[do] DOI:10.1093/rheumatology/kex293

  7 / 1084 MEDLINE  
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[PMID]: 28842398
[Au] Autor:Jennette JC; Nachman PH
[Ad] Address:Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina jcj@med.unc.edu.
[Ti] Title:ANCA Glomerulonephritis and Vasculitis.
[So] Source:Clin J Am Soc Nephrol;12(10):1680-1691, 2017 Oct 06.
[Is] ISSN:1555-905X
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis. ANCA vasculitis is most prevalent in individuals >50 years old. There are racial/ethnic and geographic influences on the prevalence, serotype frequencies, and clinicopathologic phenotypes. There is clinical, , and animal model evidence that ANCAs cause disease by activating neutrophils to attack small vessels. Immunomodulatory and immunosuppressive therapies are used to induce remission, maintain remission, and treat relapses. Over recent years, there have been major advances in optimizing treatment by minimizing toxic therapy and utilizing more targeted therapy.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 171013
[Lr] Last revision date:171013
[St] Status:In-Process
[do] DOI:10.2215/CJN.02500317

  8 / 1084 MEDLINE  
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[PMID]: 28771641
[Au] Autor:Land J; Abdulahad WH; Arends S; Sanders JF; Stegeman CA; Heeringa P; Rutgers A
[Ad] Address:Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.
[Ti] Title:Prospective monitoring of in vitro produced PR3-ANCA does not improve relapse prediction in granulomatosis with polyangiitis.
[So] Source:PLoS One;12(8):e0182549, 2017.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:OBJECTIVES: Patients with granulomatosis with polyangiitis (GPA) are prone to disease relapse. Currently, no good biomarkers are available to predict relapses in individual patients. This study aimed to determine whether patients at risk for relapse can be distinguished based on increased in vitro autoantibody production. METHODS: Eighty-four proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) positive GPA outpatients were prospectively monitored for up to two years and 32 healthy controls were included. At periodic intervals peripheral blood mononuclear cells were isolated, cultured and in vitro production of total and PR3-ANCA-specific IgG was determined. Moreover, serum ANCA titers were measured by indirect immunofluorescence. RESULTS: Sixteen patients (21%) relapsed during the follow-up period. At time of inclusion no significant differences were present for ANCA production between relapsing and non-relapsing patients. Samples before relapse exhibited increased serum ANCA titers and in vitro PR3-ANCA IgG levels compared with inclusion samples from non-relapsing patients. When evaluating changes over time, increasing serum ANCA titers were observed prior to relapse compared to a 1-year follow-up from non-relapsing patients. No significant change in in vitro PR3-ANCA levels occurred prior to relapse, compared to non-relapse patients. CONCLUSIONS: While differences were observed for the serum ANCA titer in relapsing and non-relapsing patients, monitoring in vitro PR3-ANCA IgG production does not improve relapse prediction in GPA patients.
[Mh] MeSH terms primary: Antibodies, Antineutrophil Cytoplasmic/blood
Granulomatosis with Polyangiitis/diagnosis
Granulomatosis with Polyangiitis/epidemiology
Myeloblastin/immunology
[Mh] MeSH terms secundary: Adult
Aged
Aged, 80 and over
Early Diagnosis
Female
Granulomatosis with Polyangiitis/metabolism
Humans
Male
Middle Aged
Population Surveillance
Prospective Studies
Recurrence
Sensitivity and Specificity
[Pt] Publication type:JOURNAL ARTICLE
[Nm] Name of substance:0 (Antibodies, Antineutrophil Cytoplasmic); EC 3.4.21.76 (Myeloblastin)
[Em] Entry month:1708
[Cu] Class update date: 170829
[Lr] Last revision date:170829
[Js] Journal subset:IM
[Da] Date of entry for processing:170804
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0182549

  9 / 1084 MEDLINE  
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[PMID]: 28637076
[Au] Autor:Krasselt M; Pierer M; Amann K; Bachmann A; Lindner TH
[Ad] Address:Sektion Rheumatologie, Klinik für Gastroenterologie und Rheumatologie, Department für Innere Medizin, Neurologie und Dermatologie, Universitätsklinikum Leipzig AöR.
[Ti] Title:Klinischer Verlauf einer Granulomatose mit Polyangiitis mit frühem Beginn in der Kindheit. [Clinical Course of an Early Childhood-Onset Granulomatosis with Polyangiitis].
[So] Source:Dtsch Med Wochenschr;142(12):904-908, 2017 Jun.
[Is] ISSN:1439-4413
[Cp] Country of publication:Germany
[La] Language:ger
[Ab] Abstract:A 26-year-old male patient presented with an eight-week history of unspecific symptoms such as weight loss and fever. Besides, he also suffered from haemoptysis, cough, and arthralgia. Since the age of twelve years, the patient has been treated for Wegner's granulomatosis. At the age of 20 years he received a kidney transplant which failed only four years later. The relapse we clinically suspected was confirmed by CT scan showing bilateral pulmonary manifestations. Moreover, we found highly positive antibodies against proteinase 3. After an induction therapy using Glucocorticoids and Rituximab, accompanied by plasmapheresis, the patient's clinical condition showed a marked improvement. We were able to discharge him continuing the treatment in an outpatient setting. Childhood-onset GPA is a life-threatening disease and often characterized by recurring relapses as well as a significantly reduced quality of life for the patient.
[Mh] MeSH terms primary: Granulomatosis with Polyangiitis/diagnosis
[Mh] MeSH terms secundary: Adult
Autoantibodies/blood
Biopsy
Combined Modality Therapy
Cyclophosphamide/therapeutic use
Follow-Up Studies
Granulomatosis with Polyangiitis/pathology
Granulomatosis with Polyangiitis/therapy
Humans
Kidney/pathology
Male
Methylprednisolone/therapeutic use
Myeloblastin/immunology
Plasmapheresis
Recurrence
Rituximab/therapeutic use
Tomography, X-Ray Computed
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE
[Nm] Name of substance:0 (Autoantibodies); 4F4X42SYQ6 (Rituximab); 8N3DW7272P (Cyclophosphamide); EC 3.4.21.76 (Myeloblastin); X4W7ZR7023 (Methylprednisolone)
[Em] Entry month:1708
[Cu] Class update date: 170817
[Lr] Last revision date:170817
[Js] Journal subset:IM
[Da] Date of entry for processing:170622
[St] Status:MEDLINE
[do] DOI:10.1055/s-0043-101977

  10 / 1084 MEDLINE  
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[PMID]: 28633877
[Au] Autor:Ndika J; Airaksinen L; Suojalehto H; Karisola P; Fyhrquist N; Puustinen A; Alenius H
[Ad] Address:Department of Bacteriology and Immunology, Medicum, University of Helsinki, Helsinki, Finland.
[Ti] Title:Epithelial proteome profiling suggests the essential role of interferon-inducible proteins in patients with allergic rhinitis.
[So] Source:J Allergy Clin Immunol;140(5):1288-1298, 2017 Nov.
[Is] ISSN:1097-6825
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:BACKGROUND: Seasonal allergic rhinitis (SAR) caused by intermittent exposure to seasonal pollen causes itching, nasal congestion, and repeated sneezing, with profound effects on quality of life, work productivity, and school performance. Although both the genotype and environmental factors can contribute to the immunologic basis of allergic reactions, the molecular underpinnings associated with the pathogenesis of allergic rhinitis are not entirely clear. METHODS: To address these questions, nasal epithelial brushings were collected from 29 patients with SAR and 31 control subjects during and after the pollen season. We then implemented an orbitrap-based, bottom-up, label-free quantitative proteomics approach, followed by multivariate analyses to identify differentially abundant (DA) proteins among the 4 sample groups. RESULTS: We identified a total of 133 DA proteins for which the most significantly overrepresented functional category was found to be interferon 1 signaling. Two proteins, cystatin 1 and myeloblastin, the former of which protects against protease activity of allergens and the latter with a role in epithelial barrier function, were DA in patients with SAR and control subjects, irrespective of season. Moreover, interferon-inducible protein with tetratricopeptide repeats 1, cystatin 1, and interferon-inducible protein with tetratricopeptide repeats 3 were found to be differentially regulated between patients with SAR and control subjects, with inverse abundance dynamics during the transition from fall to spring. CONCLUSION: We identified type 1 interferon-regulated proteins as biomarkers in patients with SAR, potentially playing an important role in its pathogenesis. Moreover, when compared with patients with SAR, healthy subjects exhibit an antagonistic proteomic response across seasons, which might prove to be a therapeutic target for disease prevention.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1706
[Cu] Class update date: 171107
[Lr] Last revision date:171107
[St] Status:In-Process


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BIREME/PAHO/WHO - Latin American and Caribbean Center on Health Sciences Information