VEXAS within the spectrum of rheumatologic disease

Author(s):  
Matthew J. Koster ◽  
Kenneth J. Warrington
1989 ◽  
Vol 7 (3) ◽  
pp. 629-639 ◽  
Author(s):  
James E. Brick ◽  
John F. Brick

2007 ◽  
Vol 26 (11) ◽  
pp. 1867-1870 ◽  
Author(s):  
Ismail Hanta ◽  
Suleyman Ozbek ◽  
Sedat Kuleci ◽  
Murat Sert ◽  
Ali Kocabas

2017 ◽  
Vol 41 ◽  
pp. 283.e11-283.e18 ◽  
Author(s):  
Stephanie A. Chapman ◽  
Daniel Delgadillo ◽  
Elizabeth MacGuidwin ◽  
Joshua I. Greenberg ◽  
Andrew P. Jameson

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yael Kusne ◽  
Michael Christiansen ◽  
Christopher Conley ◽  
Juan Gea-Banacloche ◽  
Ayan Sen

Background. Hemophagocytic lymphohistiocytosis (HLH) was originally described in pediatric patients presenting with fever, hepatosplenomegaly, and blood cell abnormalities. Later, HLH was recognized to occur in adults, often associated with hematologic malignancies or serious infections. Conclusion. Patients presenting with HLH are critically ill, and rapid diagnosis is key. In adults, the search for the trigger must begin promptly as time to diagnosis effects survival. The underlying trigger in our patients was Histoplasma capsulatum infection, which is rare in the southwestern United States. Prompt diagnosis led to recovery in one patient, while the other did not survive.


Author(s):  
AK Makol ◽  
B Chakravorty ◽  
MB Heller ◽  
B Riley

Research has shown hypermobility Ehlers–Danlos syndrome (hEDS) to be associated with some complicated rheumatologic disease. In this feature paper, the authors discuss the prevalence and pathophysiology of rheumatologic conditions, specifically ankylosing spondylitis and rheumatoid arthritis, in patients with hEDS. Furthermore, the authors discuss possible reasons for the association of hEDS with these rheumatologic diseases.


Author(s):  
Antoinette Pusateri ◽  
Ashley Hatcher ◽  
Nisha Patel ◽  
Joy Lehman ◽  
Alice Hinton ◽  
...  

Abstract Purpose Infliximab promotes remission in patients with inflammatory bowel disease (IBD) and rheumatologic disease (RD). Rapid infliximab infusions (RI) reduce infusion time from 2 hours to 1 hour and can enhance access to care, as defined by capacity, safety, and patient characteristics. Our hypothesis for the study described here was that use of RI can enhance access for patients. Methods Data on all patients receiving infliximab for IBD or RD at our outpatient infusion center from February 2016 to August 2017 were retrospectively analyzed. Demographic and clinical information were collected. Results Of 348 patients who received infliximab, 205 had IBD and 143 had RD. In terms of capacity, 40% of patients received RI, resulting in a 16.1% decrease in average daily infusion time and a 9.8% increase in average daily available scheduled infusion chair time (P = 0.720). In terms of safety, 4 patients switched back to standard infusions after RI, after 3 specifically had reactions to RI. In terms of patient characteristics, more patients with RD versus IBD received RI (P = 0.020). Among the patients with RD, a lower proportion receiving RI were female (P = 0.043). For the patients with IBD, a higher proportion receiving RI were white (P = 0.048). Among both patients with RD and patients with IBD, a higher proportion receiving RI had private insurance (P = 0.016 and P = 0.018, respectively). Conclusion RI were safe and increased available chair time. Females with RD, patients of non-White race with IBD, and patients with public insurance were less likely to receive RI. Future directions include patient surveys and evaluation of implicit bias against patient factors that may impact access to RI.


Hematology ◽  
2018 ◽  
Vol 2018 (1) ◽  
pp. 313-317 ◽  
Author(s):  
John M. Gansner ◽  
Nancy Berliner

Abstract Catastrophic antiphospholipid antibody syndrome (CAPS) and macrophage activation syndrome (MAS) are both life-threatening hematologic disorders that infrequently afflict patients with rheumatologic disease. CAPS is characterized by fulminant multiorgan damage related to small vessel thrombosis in the setting of persistent antiphospholipid antibodies. It can occur in patients with rheumatologic diseases such as systemic lupus erythematosus but can also affect patients who do not have rheumatologic disease. By contrast, the term MAS is applied when patients with rheumatologic disease develop hemophagocytic lymphohistiocytosis (HLH); therefore, patients with MAS have an underlying rheumatologic disease by definition. Similar to CAPS, HLH/MAS can have a fulminant presentation, but the pathogenesis and manifestations are different. In both CAPS and MAS, management generally includes but is not limited to immunosuppression with steroids. Fatalities are relatively common and morbidity is often significant. Early recognition of these disorders and initiation of timely treatment are important. More effective therapies for both syndromes are urgently needed.


Sign in / Sign up

Export Citation Format

Share Document