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1.
Mod Rheumatol ; 2024 Apr 08.
Artículo en Inglés | MEDLINE | ID: mdl-38590042

RESUMEN

OBJECTIVES: To access the real-world clinical management of physicians who treat Takayasu arteritis (TAK) and giant cell arteritis (GCA) after the publication of the Japanese Circulation Society (JCS) 2017 Guidelines for the Management of Vasculitis Syndrome. METHODS: This descriptive, cross-sectional study utilized self-administered electronic questionnaires, which were answered in February 2022 by physicians treating TAK or GCA and registered with Macromill Inc. RESULTS: The 329 survey respondents comprised 110 cardiologists, 110 rheumatologists, 34 cardiovascular surgeons, 24 surgeons, 35 internal medicine physicians, 13 nephrologists, and 7 pediatricians. The 2017 JCS Guidelines were the most commonly referenced information source for resolving clinical questions, accessed by 70% of respondents. Ophthalmoscopy was performed in only 50% of patients with TAK, and in 70% for GCA. The median percentages of patients who underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography for TAK and GCA patients were 23% and 20% at diagnosis, respectively, and 10% each at follow-up within 12 months. Tocilizumab was the most frequently used medication in combination with glucocorticoids for both TAK and GCA, especially in remission induction therapy for relapsed patients. CONCLUSIONS: The majority of physician treating TAK and GCA referred to the 2017 JCS guidelines. This report clarified the current clinical practice for large vessel vasculitis in Japan, providing information for the next revision of the guidelines.

2.
Artículo en Inglés | MEDLINE | ID: mdl-38608193

RESUMEN

OBJECTIVES: To evaluate the effectiveness and safety of two different intravenous methylprednisolone (IVMP) pulse doses in patients with severe microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). METHODS: We emulated a target trial using observational data from the nationwide registry in Japan. Patients with severe glomerulonephritis or diffuse alveolar haemorrhage were selected and pseudo-randomised into three groups using propensity score-based overlap weighting as follows: non-IVMP, IVMP 0.5 g/day, and IVMP 1.0 g/day. The primary outcome was all-cause death, and the secondary outcomes were composite all-cause death and kidney failure, severe relapse, and serious infection from 2 to 48 weeks after treatment initiation. To estimate the treatment effects, the Cox proportional hazard model and Fine-Gray subdistribution hazard model were used. RESULTS: In this emulated target trial, of 201 eligible patients (MPA, 175; GPA, 26), 6 (2.8%) died, 4 (2.0%) had kidney failure, 11 (5.3%) had severe relapse, and 40 (19.8%) had severe infections. Hazard ratios (HR) for IVMP 0.5 g/day and IVMP 1.0 g/day pulse groups compared with non-IVMP pulse were as follows: all-cause death = 0.46 (95% confidence interval [95%CI]: 0.07-2.81) and 0.07 (95%CI: 0.01-0.41); all-cause death/kidney failure = 1.18 (95%CI: 0.26-5.31) and 0.59 (95%CI: 0.08-4.52); subdistribution HRs for severe relapse = 1.26 (95%CI: 0.12-13.70) and 3.36 (95%CI: 0.49-23.29); and serious infection = 1.88 (95%CI: 0.76-4.65) and 0.94 (95%CI: 0.28-3.13). CONCLUSIONS: IVMP 1.0 g/day pulse may improve 48-week mortality in patients with severe MPA/GPA.

3.
Arthritis Rheumatol ; 76(7): 1120-1129, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38343337

RESUMEN

OBJECTIVE: We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN). METHODS: Patients with PAN were recruited between 1990 and 2020 from observational cohorts of nine countries across Europe, Japan, and North America. Eligibility was retrospectively defined using the European Medicines Agency classification algorithm. Patients with PAN related to hepatitis B virus (n = 12) and two monogenic diseases mimicking PAN, deficiency of adenosine deaminase 2 enzyme (n = 16) or familial Mediterranean fever (n = 11), were excluded. Data regarding organ involvement, relapse, disease-related damage, and survival were analyzed. RESULTS: Three hundred fifty-eight patients (female:male ratio 174:184), including those with systemic PAN (sPAN, n = 282) and cutaneous PAN (n = 76), were included. Twenty-five were pediatric onset. Mean ± SD age at diagnosis was 44.3 ± 18.1 years. Constitutional symptoms (71.5%), cutaneous involvement (70.5%), musculoskeletal findings (69.1%), and neurologic features (48.0%) were common manifestations. Among patients with sPAN, gastrointestinal involvement and proteinuria over 400 mg/day were reported in 52.2% and 11.2%, respectively. During a median (interquartile range) 59.6 (99.5) months of follow-up, relapse occurred in 48.5% of patients. One, 5- and 10-year survival rates for sPAN were 97.1%, 94.0%, and 89.0%, respectively. Predictors of death for sPAN included age ≥65 years at diagnosis, serum creatinine at diagnosis >140 µmol/L, gastrointestinal manifestations, and central nervous system (CNS) involvement. CONCLUSION: The spectrum of PAN remains a complex, multifaceted disease. Relapse is common. Age ≥65 years and serum creatinine >140 µmol/L at diagnosis, as well as gastrointestinal and CNS involvement, are independent predictors of death in sPAN.


Asunto(s)
Poliarteritis Nudosa , Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Europa (Continente)/epidemiología , Anciano , América del Norte/epidemiología , Japón/epidemiología , Adulto Joven , Proteinuria/etiología , Recurrencia , Tasa de Supervivencia
4.
Artículo en Inglés | MEDLINE | ID: mdl-38036300

RESUMEN

Cryopyrin-associated periotic syndrome (CAPS) is a rare autoinflammatory disease (AID) caused by genetic variants in innate immunity genes. AIDs, including CAPS, mediate proinflammatory cytokines such as interleukin (IL)-1 and IL-18 and result in severe systemic inflammation. A gain-of-function mutation in the NLRP3 gene, which encodes the protein cryopyrin, was identified to be responsible for CAPS in 2001, and since then several additional pathogenic mutations have been found. Moreover, other phenotypes have been identified based on severity and symptomatology, including familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID)/chronic neurologic cutaneous articular syndrome (CINCA). Prompt diagnosis of CAPS remains challenging, however, due to unspecific, extensive clinical signs, and delayed diagnosis and treatment targeting IL-1 lead to multiorgan damage. Another factor complicating diagnosis is the existence of somatic mosaic mutations in the NLRP3 gene in some cases, resulting in symptoms and clinical courses that are atypical. The frequency of somatic mosaic mutations in CAPS was estimated to be 19% in a systematic review. Psoriasis is a chronic inflammatory skin disease that affects about 3% of the global population. Although no reports have shown complication between CAPS and psoriasis, these diseases have several similarities and potential relationships, for instance activation of Th17 cells in the dermis and increased NLRP3 gene expression in psoriatic skin compared with normal skin. Here we report a case of CAPS due to a somatic mosaic mutation with recurrent circinate erythematous psoriasis.

5.
Mod Rheumatol ; 2023 Oct 06.
Artículo en Inglés | MEDLINE | ID: mdl-37801552

RESUMEN

OBJECTIVES: To identify the optimal dose of intravenous cyclophosphamide (IVCY) for induction therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: We retrospectively assessed patients with AAV who received IVCY every 2-3 weeks during the remission induction phase. The associations of the IVCY dose with infection-free survival and relapse-free survival were analysed using a Cox regression model. We compared patients in three categories: very low-dose (VLD), low-dose (LD), and conventional dose (CD) (<7.5 mg/kg, 7.5-12.5 mg/kg, and >12.5 mg/kg, respectively). The non-linear association between IVCY dose and the outcomes were also evaluated. RESULTS: Of the 80 patients (median age 72 years), 12, 42, and 26 underwent the VLD, LD, and CD regimens, respectively, of whom 4, 3, and 7 developed infection or died. The adjusted hazard ratios for infection or death were 4.3 (95% confidence interval (CI) 0.94-19.8) for VLD and 5.1 (95% CI 1.21-21.3) for CD, compared with LD. We found the hazard ratio for infection or death increased when the initial IVCY dose exceeded 9 mg/kg. Relapse-free survival did not differ clearly. CONCLUSION: Low-dose IVCY (7.5-12.5 mg/kg) may result in fewer infections and similar relapse rates compared with the conventional regimen (>12.5 mg/kg).

6.
Heliyon ; 9(8): e19072, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-37636359

RESUMEN

In complement activation system, although the classical pathway has shown to play a critical role for the pathogenesis of SLE, the role of lectin pathway has remained unknown in the pathogenesis of SLE. As Mannose-binding lectin-associated serine proteases (MASPs) are associated with activation of the lectin pathway, we conducted this study to clarify MASPs associations in the pathogenesis of SLE. We evaluated the serum level of MASPs (MASP-1 and MASP-2) in total 68 SLE patients consisting of 15 patients with biopsy-confirmed membranous lupus nephritis (M-LN), 35 patients with biopsy-confirmed proliferative lupus nephritis (P-LN), and 18 SLE patients without LN (non-LN). Our data showed that the serum levels of MASPs were reduced in both P-LN and non-LN although those of M-LN were not reduced. Our data show that the lectin pathway mediated by MASPs plays a critical role for the pathogenesis of SLE except for M-LN.

7.
Clin Nucl Med ; 48(10): 890-893, 2023 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-37486305

RESUMEN

ABSTRACT: Lupus enteritis is classified into the colon poly-ulcerative type and the small intestine ischemic serositis type. Colon poly-ulcerative lupus enteritis is a disease that is mainly due to mesenteric arteritis. In recent years, 18 F-FDG PET/CT has been frequently used to assess the extent of the disease in patients with systemic vasculitis. We present the case report of 18 F-FDG PET/CT results in a 57-year-old woman with colon poly-ulcerative lupus enteritis.


Asunto(s)
Colitis Ulcerosa , Enteritis , Femenino , Humanos , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , Fluorodesoxiglucosa F18 , Inflamación , Tomografía de Emisión de Positrones , Enteritis/complicaciones , Enteritis/diagnóstico por imagen
8.
Mod Rheumatol Case Rep ; 8(1): 125-132, 2023 Dec 29.
Artículo en Inglés | MEDLINE | ID: mdl-37319013

RESUMEN

In response to the coronavirus disease 2019 pandemic, the coronavirus disease 2019 vaccine was rapidly developed and the effectiveness of the vaccine has been established. However, various adverse effects have been reported, including the development of autoimmune diseases. We report a case of new-onset polyarteritis nodosa in a 32-year-old male following the coronavirus disease 2019 vaccination. The patient developed limb pain, fever, pulmonary embolism, multiple subcutaneous nodules, and haematomas. Skin biopsy revealed necrotising inflammation accompanied by fibrinoid necrosis and high inflammatory cell infiltration in the walls of medium to small arteries. The symptoms resolved following corticosteroid treatment. Although it is difficult to prove a relationship between the vaccine and polyarteritis nodosa, similar cases have been reported and further reports and analyses are therefore necessary.


Asunto(s)
Vacunas contra la COVID-19 , COVID-19 , Poliarteritis Nudosa , Adulto , Humanos , Masculino , Corticoesteroides , COVID-19/diagnóstico , COVID-19/prevención & control , COVID-19/complicaciones , Vacunas contra la COVID-19/efectos adversos , Poliarteritis Nudosa/diagnóstico , Poliarteritis Nudosa/etiología , Piel/patología
9.
J Rheumatol ; 50(9): 1152-1158, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37263656

RESUMEN

OBJECTIVE: To clarify seasonal and other environmental effects on the onset of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We enrolled patients with new-onset eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and granulomatosis with polyangiitis (GPA) registered in the database of a Japanese multicenter cohort study. We investigated the relationship between environmental factors and clinical characteristics. Seasons were divided into 4 (spring, summer, autumn, and winter), and the seasonal differences in AAV onset were analyzed using Pearson chi-square test, with an expected probability of 25% for each season. RESULTS: A total of 454 patients were enrolled, with a mean age of 70.9 years and a female proportion of 55.5%. Overall, 74, 291, and 89 patients were classified as having EGPA, MPA, and GPA, respectively. Positivity for myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA was observed in 355 and 46 patients, respectively. Overall, the seasonality of AAV onset significantly deviated from the expected 25% for each season (P = 0.001), and its onset was less frequently observed in autumn. In ANCA serotypes, seasonality was significant in patients with MPO-ANCA (P < 0.001), but not in those with PR3-ANCA (P = 0.97). Additionally, rural residency of patients with AAV was associated with PR3-ANCA positivity and biopsy-proven pulmonary vasculitis. CONCLUSION: The onset of AAV was influenced by seasonal variations and was less frequently observed in autumn. In contrast, the occurrence of PR3-ANCA was triggered, not by season, but by rural residency.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Síndrome de Churg-Strauss , Granulomatosis con Poliangitis , Poliangitis Microscópica , Humanos , Femenino , Anciano , Granulomatosis con Poliangitis/complicaciones , Anticuerpos Anticitoplasma de Neutrófilos , Estaciones del Año , Síndrome de Churg-Strauss/complicaciones , Estudios Retrospectivos , Estudios de Cohortes , Japón/epidemiología , Mieloblastina , Poliangitis Microscópica/complicaciones , Peroxidasa
11.
Rheumatology (Oxford) ; 62(12): 3924-3931, 2023 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-36961329

RESUMEN

OBJECTIVES: To investigate the association between decreased serum IgG levels caused by remission-induction immunosuppressive therapy of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the development of severe infections. METHODS: We conducted a retrospective cohort study of patients with new-onset or severe relapsing AAV enrolled in the J-CANVAS registry, which was established at 24 referral sites in Japan. The minimum serum IgG levels up to 24 weeks and the incidence of severe infection up to 48 weeks after treatment initiation were evaluated. After multiple imputations for all explanatory variables, we performed the multivariate analysis using a Fine-Gray model to assess the association between low IgG (the minimum IgG levels <500 mg/dl) and severe infections. In addition, the association was expressed as a restricted cubic spline (RCS) and analysed by treatment subgroups. RESULTS: Of 657 included patients (microscopic polyangiitis, 392; granulomatosis with polyangiitis, 139; eosinophilic granulomatosis with polyangiitis, 126), 111 (16.9%) developed severe infections. The minimum serum IgG levels were measured in 510 patients, of whom 77 (15.1%) had low IgG. After multiple imputations, the confounder-adjusted hazard ratio of low IgG for the incidence of severe infections was 1.75 (95% confidence interval: 1.03-3.00). The RCS revealed a U-shaped association between serum IgG levels and the incidence of severe infection with serum IgG 946 mg/dl as the lowest point. Subgroup analysis showed no obvious heterogeneity between treatment regimens. CONCLUSION: Regardless of treatment regimens, low IgG after remission-induction treatment was associated with the development of severe infections up to 48 weeks after treatment initiation.


Asunto(s)
Agammaglobulinemia , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Síndrome de Churg-Strauss , Granulomatosis con Poliangitis , Poliangitis Microscópica , Humanos , Granulomatosis con Poliangitis/tratamiento farmacológico , Estudios Retrospectivos , Agammaglobulinemia/inducido químicamente , Quimioterapia de Inducción , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Poliangitis Microscópica/tratamiento farmacológico , Inmunoglobulina G/uso terapéutico , Anticuerpos Anticitoplasma de Neutrófilos
12.
Immun Inflamm Dis ; 11(2): e777, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36840498

RESUMEN

OBJECTIVES: Lupus cystitis is a rare but serious complication of systemic lupus erythematosus (SLE) that can cause permanent bladder dysfunction, leading to irreversible deterioration of kidney function. We report two cases of SLE with lupus cystitis who showed different images from the same cause of disease. METHODS: Patient 1, a 67-year-old woman diagnosed with SLE presented with persistent dysuria for 3 weeks with sudden headache and vomiting. She was hospitalized because of acute kidney injury; the serum creatinine level was 10.68 mg/dL. Computed tomography (CT) showed significant bilateral ureteral stenosis and bilateral hydronephrosis. Patient 2, a 45-year-old woman diagnosed with SLE presented with dysuria requiring self-catheterization. CT showed significant bilateral ureteral dilatation and bilateral hydronephrosis. RESULTS: In patient 1, the right kidney was afunctional. Left nephrostomy was performed on Day 2. Her serum creatinine returned to the normal range. In patient 2, After admission, she changed to an indwelling bladder catheter. Her serum creatinine level improved from 2.04 to 1.31 mg/dL. CONCLUSION: In patients with lupus cystitis, the urinary tract is commonly dilated, but stenosis has been seen in rare case. Physicians should be careful in diagnosing it.


Asunto(s)
Cistitis , Hidronefrosis , Lupus Eritematoso Sistémico , Sistema Urinario , Humanos , Femenino , Anciano , Persona de Mediana Edad , Disuria/complicaciones , Constricción Patológica/complicaciones , Creatinina , Hidronefrosis/complicaciones
13.
Sci Rep ; 13(1): 2509, 2023 02 13.
Artículo en Inglés | MEDLINE | ID: mdl-36782006

RESUMEN

IgG4-related disease (IgG4-RD) was recently described in Japan. It is characterised by extensive organ involvement with tissue fibrosis. We assessed the performance of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria as well as differences between patients with and without IgG4-RD. In this retrospective, single-centre study of 50 patients admitted with suspected IgG4-RD, we evaluated the sensitivity and specificity of both criteria. We also compared clinical characteristics and laboratory data of patients with IgG4-RD (n = 42) and patients without IgG4-RD (n = 8). The ACR/EULAR classification criteria had 88.1% sensitivity and 87.5% specificity for IgG4-RD diagnosis. The RCD criteria had 100% sensitivity and 50% specificity. Patients with IgG4-RD had significantly more affected organs (p = 0.002). Patients with a single affected organ and IgG4-RD had significantly higher serum IgG4/IgG ratios (p = 0.027), lower serum C-reactive protein levels (p = 0.020), and lower total haemolytic complement activity (p = 0.044) than those without IgG4-RD. The ACR/EULAR classification criteria have high specificity and the RCD criteria have high sensitivity for diagnosing IgG4-RD. The number of affected organs is important for diagnosing IgG4-RD.


Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Enfermedades Reumáticas , Reumatología , Humanos , Estados Unidos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Estudios Retrospectivos , Sensibilidad y Especificidad
14.
Clin Nucl Med ; 48(3): 276-277, 2023 Mar 01.
Artículo en Inglés | MEDLINE | ID: mdl-36638239

RESUMEN

ABSTRACT: 18 F-FDG PET/CT is increasingly being used in the diagnosis of systemic vasculitis syndromes, especially large-vessel vasculitis. We present a case of giant cell arteritis in an 84-year-old man who exhibited large-vessel lesions in the periphery but none in the trunk. This case highlights the usefulness of PET/CT for revealing localized inflammation.


Asunto(s)
Arteritis de Células Gigantes , Masculino , Humanos , Anciano de 80 o más Años , Arteritis de Células Gigantes/complicaciones , Arteritis de Células Gigantes/diagnóstico por imagen , Arteritis de Células Gigantes/patología , Tomografía Computarizada por Tomografía de Emisión de Positrones , Fluorodesoxiglucosa F18 , Arterias , Inflamación/diagnóstico por imagen , Extremidad Inferior/patología , Radiofármacos
15.
Mod Rheumatol ; 33(6): 1110-1116, 2023 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-36469011

RESUMEN

OBJECTIVES: This study was conducted to determine autoantibodies associated with lupus nephritis (LN), especially those useful in diagnosing proliferative and membranous nephritis. METHODS: A total of 106 patients with LN and 63 patients with systemic lupus erythematosus but no nephritis were enrolled; then, 55 patients were selected from the LN group and were divided into two groups: proliferative nephritis patients (n = 36) and membranous nephritis patients (n = 19). The autoantibody profiles of patients' sera were evaluated using the EUROLINE ANA Profile 3 (IgG) kit. RESULTS: A higher positivity rate of anti-double-stranded DNA antibody and anti-histone antibody was seen in LN patients compared to nonrenal systemic lupus erythematosus patients. In comparing between proliferative and membranous nephritis, the positivity of anti-nucleosome antibody was higher in proliferative nephritis, although it was not statistically significant. However, anti-nucleosome antibody-positive patients with LN had a higher prevalence of haematuria and pyuria, which are strong indications of proliferative nephritis. Also, a significantly higher positivity rate of anti-RNP70 antibody was seen in membranous nephritis compared to proliferative nephritis. CONCLUSIONS: Our results showed that anti-nucleosome and anti-RNP70 antibodies may be predictive nonhistological factors for discriminating between proliferative and membranous LN.


Asunto(s)
Glomerulonefritis Membranosa , Lupus Eritematoso Sistémico , Nefritis Lúpica , Humanos , Nefritis Lúpica/diagnóstico , Autoanticuerpos , Nucleosomas
16.
Rheumatology (Oxford) ; 62(7): 2525-2533, 2023 07 05.
Artículo en Inglés | MEDLINE | ID: mdl-36326436

RESUMEN

OBJECTIVES: Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive DM is characterized by rapidly progressive interstitial lung disease and has a poor prognosis. We aimed to investigate whether anti-MDA5 antibody titres and cytokine levels predict clinical course, and evaluate changes in both parameters before and after diagnosis. METHODS: This was a retrospective, single-centre study in 38 patients with anti-MDA5 antibody-positive DM. We compared clinical characteristics and laboratory data at diagnosis between patients in the treatment response (n = 23) and non-response (n = 15) groups, and between those in the relapse (n = 5) and non-relapse (n = 24) groups. We also measured serum anti-MDA5 antibody titres and cytokine levels before and after diagnosis. RESULTS: The non-response group was older, had a higher ground-glass opacity score, lower PaO2/FiO2, higher CRP level, and higher anti-MDA5 antibody titre than the response group. No cytokines significantly differed between groups at diagnosis. The relapse group had a significantly higher anti-MDA5 antibody titre than the non-relapse group. In the survivor group, the anti-MDA5 antibody titre and levels of IFN-α, IFN-γ, monocyte chemotactic protein-1 (MCP-1), IL-6, IL-33, CRP, and ferritin were significantly lower 6 months post-treatment than at diagnosis. Macrophage-associated cytokines such as IL-6, IL-8, IL-18 and MCP-1 increased after anti-MDA5 antibody positivity in three patients who were anti-MDA5 antibody-positive before diagnosis. CONCLUSION: The anti-MDA5 antibody titre at diagnosis may predict the clinical course. Levels of macrophage-associated cytokines significantly declined at 6 months post-treatment, and they may have increased after anti-MDA5 antibody titre positivity.


Asunto(s)
Citocinas , Dermatomiositis , Humanos , Pronóstico , Interleucina-6 , Estudios Retrospectivos , Helicasa Inducida por Interferón IFIH1 , Enfermedad Crónica , Autoanticuerpos , Progresión de la Enfermedad
17.
Arthritis Res Ther ; 24(1): 204, 2022 08 23.
Artículo en Inglés | MEDLINE | ID: mdl-35999568

RESUMEN

BACKGROUND: This study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan. METHODS: We analyzed the clinical information of 663 Asian patients with AAV (total AAV), including 558 patients with newly diagnosed AAV and 105 with relapsed AAV. Clinical findings were compared between patients with and without HP. To elucidate the relevant manifestations for HP development, multivariable logistic regression analyses were additionally performed. RESULTS: Of the patients with AAV (mean age, 70.2 ± 13.5 years), HP was noted in 30 (4.52%), including 20 (3.58%) with newly diagnosed AAV and 10 (9.52%) with relapsed AAV. Granulomatosis with polyangiitis (GPA) was classified in 50% of patients with HP. A higher prevalence of GPA was significantly observed in patients with HP than in those without HP in total AAV and newly diagnosed AAV (p < 0.001). In newly diagnosed AAV, serum proteinase 3 (PR3)-ANCA positivity was significantly higher in patients with HP than in those without HP (p = 0.030). Patients with HP significantly had ear, nose, and throat (ENT) (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.03-2.14, p = 0.033) and mucous membrane/eye manifestations (OR 5.99, 95% CI 2.59-13.86, p < 0.0001) in total AAV. Moreover, they significantly had conductive hearing loss (OR 11.6, 95% CI 4.51-29.57, p < 0.0001) and sudden visual loss (OR 20.9, 95% CI 5.24-85.03, p < 0.0001). CONCLUSION: GPA was predominantly observed in patients with HP. Furthermore, in newly diagnosed AAV, patients with HP showed significantly higher PR3-ANCA positivity than those without HP. The ear and eye manifestations may be implicated in HP development.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Granulomatosis con Poliangitis , Meningitis , Anciano , Anciano de 80 o más Años , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/epidemiología , Anticuerpos Anticitoplasma de Neutrófilos , Estudios Transversales , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/epidemiología , Humanos , Hipertrofia , Japón/epidemiología , Meningitis/epidemiología , Persona de Mediana Edad , Mieloblastina , Peroxidasa , Estudios Retrospectivos
18.
Ther Apher Dial ; 26(3): 515-521, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35247214

RESUMEN

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is associated with high mortality. We examined the efficacy of plasma exchange (PE) therapy for reducing mortality in patients with patients with microscopic polyangiitis (MPA) and DAH. METHODS: In this 52-week, nonrandomized, open-label, one-arm, historical control, double-center controlled trial, four patients with MPA and DAH admitted to Juntendo University Hospital or Juntendo Koto Geriatric Medical Center between 2018 and 2021 were enrolled. Sixteen patients with MPA and DAH admitted to the two centers between 1998 and 2018 who did not receive PE were included as the historical control group. The primary outcome was the 52-week survival rate of patients in each treatment arm. RESULTS: The 52-week survival rate of patients in the PE group (n = 4) was higher than that of the historical control group (n = 16) (100% vs. 13%, p = 0.04). CONCLUSION: We found that PE may be efficacious for reducing mortality in patients with MPA and DAH.


Asunto(s)
Enfermedades Pulmonares , Poliangitis Microscópica , Anciano , Hemorragia/etiología , Hemorragia/terapia , Hospitalización , Humanos , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/terapia , Poliangitis Microscópica/complicaciones , Poliangitis Microscópica/terapia , Intercambio Plasmático , Alveolos Pulmonares
19.
Dalton Trans ; 51(4): 1571-1579, 2022 Jan 25.
Artículo en Inglés | MEDLINE | ID: mdl-34990504

RESUMEN

This work introduces a novel route to perovskite LaTiO2N nanopowders (NPs) via nitridation of perovskite LaTiO3 NPs in an NH3 gas flow at 1050 °C/NH3/15 h, in which a simple perovskite structure (ABX3) is retained during nitridation. The LaTiO3 NP is formed with a trace of a second phase in a precursor oxide NP (LTO-4/3) produced using liquid-feed flame spray pyrolysis (LF-FSP) of a metallo-organic ethanol solution with La/Ti = 4/3. The characterization of the resulting powders allows for a comparison with LaTiO2N NPs synthesized by the nitridation of the La2Ti2O7 precursor oxide NP (LTO-1) with a perovskite slab structure (A2B2X7) also prepared by LF-FSP of a La/Ti = 1 solution. Williamson-Hall plots suggest that the as-produced LaTiO2N from LTO-1 offers a quite small but effective crystallite size of 16-18 nm with almost no lattice spacing fluctuations, while LaTiO2N from LTO-4/3 presents a larger effective crystallite size of 50-52 nm with some lattice spacing fluctuations. UV-vis diffuse reflectance analysis reveals that, unlike LaTiO2N from LTO-1, the spectra of LaTiO2N from LTO-4/3 show a quite low absorption background above the wavelength of the optical absorption edge (∼580 nm), suggesting good crystallinity with a very low surface defect density. Both oxynitride NPs appear to offer utility as inorganic pigments with different colours, while LaTiO2N NPs from LTO-4/3 have advantages for various applications, including potential as a visible-light-driven water splitting photocatalyst.

20.
Lupus ; 30(14): 2292-2297, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34693822

RESUMEN

BACKGROUND: In patients with systemic lupus erythematosus (SLE), infections, especially bacteremia, can occur throughout the course of the disease and are often fatal. We evaluated the characteristics of patients with bacteremia and SLE. METHODS: In this retrospective single-center observational study, we analyzed bacteremia in 65 patients with SLE. We compared the group that survived to the group that died. To compare demographic and clinical characteristics between groups, the Mann-Whitney U test was used for non-normally distributed variables. Categorical variables were compared using Fisher's exact test. RESULTS: The median observation period was 39 (interquartile range: 6-74) months. The median age was 54 (43-64) years. Patients consisted of six males and 59 females. In 49 cases, the patient survived. In 16 cases, the patient died. The dead group was older, with lower Glasgow Coma Scale scores, higher sequential organ failure assessment (SOFA) scores, and lower fibrinogen levels. CONCLUSION: When physicians encounter patients with suspected bacteremia, they should pay attention to the consciousness assessment and SOFA score, and be aware of infections caused by common microorganisms and opportunistic infections.


Asunto(s)
Bacteriemia , Lupus Eritematoso Sistémico , Bacteriemia/epidemiología , Femenino , Humanos , Japón/epidemiología , Lupus Eritematoso Sistémico/complicaciones , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo
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