scholarly journals Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy

2009 ◽  
Vol 124 (6) ◽  
pp. 1319-1325.e3 ◽  
Author(s):  
Princess U. Ogbogu ◽  
Bruce S. Bochner ◽  
Joseph H. Butterfield ◽  
Gerald J. Gleich ◽  
Johannes Huss-Marp ◽  
...  
Leukemia ◽  
2021 ◽  
Author(s):  
Shinsuke Hirabayashi ◽  
Ellie R. Butler ◽  
Kentaro Ohki ◽  
Nobutaka Kiyokawa ◽  
Anke K. Bergmann ◽  
...  

Author(s):  
Andrea Frustaci ◽  
Maria Alfarano ◽  
Romina Verardo ◽  
Chiara Agrati ◽  
Rita Casetti ◽  
...  

Abstract Aims  Necrotizing coronary vasculitis (NCV) is a rare entity usually associated to myocarditis which incidence, cause, and response to therapy is unreported. Methods and results  Among 1916 patients with biopsy-proven myocarditis, 30 had NCV. Endomyocardial samples were retrospectively investigated with immunohistochemistry for toll-like receptor 4 (TLR4) and real-time polymerase chain reaction (PCR) for viral genomes. Serum samples were processed for anti-heart autoantibodies (Abs), IL-1β, IL-6, IL-8, tumour necrosis factor (TNF)-α. Identification of an immunologic pathway (including virus-negativity, TLR4-, and Ab-positivity) was followed by immunosuppression. Myocarditis-NCV cohort was followed for 6 months with 2D-echo and/or cardiac magnetic resonance and compared with 60 Myocarditis patients and 30 controls. Increase in left ventricular ejection fraction ≥10% was classified as response to therapy. Control endomyocardial biopsy followed the end of treatment. Twenty-six Myocarditis-NCV patients presented with heart failure; four with electrical instability. Cause of Myocarditis-NCV included infectious agents (10%) and immune-mediated causes (chest trauma 3%; drug hypersensitivity 7%; hypereosinophilic syndrome 3%; primary autoimmune diseases 33%, idiopathic 44%). Abs were positive in immune-mediated Myocarditis-NCV and virus-negative Myocarditis; Myocarditis-NCV patients with Ab+ presented autoreactivity in vessel walls. Toll-like receptor 4 was overexpressed in immune-mediated forms and poorly detectable in viral. Interleukin-1β was significantly higher in Myocarditis-NCV than Myocarditis, the former presenting 24% in-hospital mortality compared with 1.5% of Myocarditis cohort. Immunosuppression induced improvement of cardiac function in 88% of Myocarditis-NCV and 86% of virus-negative Myocarditis patients. Conclusion  Necrotizing coronary vasculitis is histologically detectable in 1.5% of Myocarditis. Necrotizing coronary vasculitis includes viral and immune-mediated causes. Intra-hospital mortality is 24%. The immunologic pathway is associated with beneficial response to immunosuppression.


2017 ◽  
Vol 44 (11) ◽  
pp. 1612-1618 ◽  
Author(s):  
Niharika Sharma ◽  
Michael S. Putman ◽  
Rekha Vij ◽  
Mary E. Strek ◽  
Anisha Dua

Objective.Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases.Methods.A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids.Results.Thirty-one of 54 patients (57%) responded to conventional treatment based on the predefined variables of improvement in myositis and/or ILD. Patients with polymyositis (PM)-ILD were more likely to respond to conventional treatment than those with dermatomyositis (DM)-ILD (67% vs 35%, p = 0.013). Twenty-three patients failed conventional treatment, 18 of whom subsequently received adjunctive tacrolimus. Ninety-four percent had improvements in ILD and 72% showed improvement in both myositis and ILD. The mean doses of prednisone decreased from baseline by 65% at 3–6 months (p = 0.002) and 81% at 1 year (p < 0.001).Conclusion.Patients with PM-ILD were more likely to respond to conventional treatment than patients with DM-ILD, but clinical characteristics and serology did not otherwise predict response to therapy. A majority of patients with MA-ILD refractory to conventional therapy improved while receiving tacrolimus and were able to decrease their dose of both glucocorticoids and other disease-modifying antirheumatic drugs.


2020 ◽  
Vol 73 (7) ◽  
pp. 1397-1401
Author(s):  
Oleksandra Ya. Pryshliak ◽  
Tetiana O. Nikiforova ◽  
Zoriana R. Tylishchak ◽  
Oleksandr P. Boichuk ◽  
Nadiya V. Vaskul ◽  
...  

The aim: To perform a retrospective analysis of leptospirosis morbidity in Ivano-Frankivsk region, to give the etiological characteristics of leptospirosis according to clinicallaboratory parameters, to determine the peculiarities of the course, prognostic features. Materials and methods: Data of the State Enterprise “Ivano-Frankivsk Regional Laboratory Center of the Ministry of Health of Ukraine”, reports and abstracts of medical records of the Regional Clinical Infectious Diseases Hospital for 2009-2018 were used. Clinical observation of patients, analysis of general-clinical, biochemical, and serological indices were carried out. Results: The leptospirosis morbidity in Ivano-Frankivsk region during 2009-2018 was higher than in general in Ukraine. Activation of foci of leptospirosis L. pomona and grippotyphosa in 2009-2013, decrease of L. grippotyphosa and increase of L. pomona foci and “new” L. autumnalis, australis, bataviae, cynopteri in 2014-2018 were noted. The emergence of leptospirosis L. autumnalis, which had a high virulence and caused a severe course, was noted. Conclusions: The leptospirosis morbidity in the Ivano-Frankivsk region over the past 10 years has exceeded the incidence in Ukraine and varied within 0.62-2.2 per 100,000. During the last 5 years, the serological structure has changed: L. pomona (27.7%) prevailed, L. grippotyphosa decreased (2.1%), L. bataviae and cynopteri, autumnalis increased (by 8.5%). Seasonality is shifted in the autumn-winter period (November-February) (L. grippotyphosa, australis, pomona, canicola). According to forecasts, leptospirosis induced by L. autumnalis was most adverse. Leptospiroses of serogroups L. australis, bataviae, cynopteri had a mild course, there were fewer complications.


1995 ◽  
Vol 13 (5) ◽  
pp. 1123-1128 ◽  
Author(s):  
C L Vogel ◽  
J Schoenfelder ◽  
I Shemano ◽  
D F Hayes ◽  
R A Gams

PURPOSE Scintigraphic flare in association with response to therapy has been well described in the medical literature. During the course of a recent breast cancer trial, it became apparent that several patients with worsening bone scan but no other clinical evidence of disease progression might have potentially benefited from continued therapy, but had therapy discontinued. A retrospective analysis of this issue was performed to assess the magnitude and scope of this problem. MATERIALS AND METHODS A total of 648 patients with hormone receptor-positive or unknown advanced breast cancer were treated as part of a large-scale trial of first-line hormonal therapy. Patients were assessed for response to therapy, including response duration, progression-free interval (PFI), overall survival, and quality of life. The retrospective analysis presented here was performed to assess whether patients with a possible scintigraphic flare within the first 16 weeks of therapy might have had therapy discontinued prematurely due to a worsening bone scan attributable to tumor flare, rather than due to disease progression. RESULTS Analysis of the hormonal trial showed that of 376 assessable patients 108 (29%) with bone disease had a possible scintigraphic flare by week 8 or 16 of the trial, based on data on the case report forms and radiology reports (bone scans and x-rays). Of these, 69 patients (64%) were continued on study therapy, which resulted in clinical benefit in 50 (72%) of those patients. In contrast, 39 patients (36%) with possible scintigraphic flare were removed from the trial. CONCLUSION We conclude that changes in bone scintigraphy that mimic progressive disease early in the course of hormonal treatment of patients with breast cancer metastatic to bone may represent scintigraphic flare associated with response. Thus, clinicians must be cognizant of the phenomenon of scintigraphic flare to avoid premature discontinuation of a potentially beneficial treatment.


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