primary antiphospholipid syndrome
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2022 ◽  
pp. 153857442110726
Author(s):  
Javad Jalili ◽  
Ramin Pourghorban ◽  
Masoud Mahmoudpour ◽  
Ali Akhavi Milani

Antiphospholipid syndrome is an autoimmune condition characterized by arteriovenous thromboembolic events. Thrombocytopenia is a common finding among these patients and is typically of mild severity not requiring any treatment. However, severe cases of thrombocytopenia should be treated. Steroids, intravenous immune globulin (IVIG), and immunomodulatory agents are the first-line treatment options, and surgical splenectomy is usually reserved for more severe and refractory cases of thrombocytopenia. Herein, we report the case of a 30-year-old man with primary antiphospholipid syndrome and severe thrombocytopenia. The patient’s thrombocytopenia had been refractory to almost all the medical managements, and surgical splenectomy could not be an option due to the patient’s high-risk condition for surgery. The patient was successfully managed by partial splenic embolization (PSE) which was a unique application of this technique.


2022 ◽  
Vol 209 ◽  
pp. 69-74
Author(s):  
Tommaso Bucci ◽  
Paul R.J. Ames ◽  
Massimo Triggiani ◽  
Roberta Parente ◽  
Antonio Ciampa ◽  
...  

Immuno ◽  
2021 ◽  
Vol 1 (4) ◽  
pp. 529-544
Author(s):  
Jacques-Olivier Pers ◽  
Hajar Bahane ◽  
Christelle Le Dantec ◽  
Nathan Foulquier ◽  
Marta E. Alarcon-Riquelme ◽  
...  

Further observations support the role of environmental factors in autoimmune diseases via the alteration of the epigenetic machinery. In this context, ultraviolet light, smoking, chemicals, and psychological stress have been described as likely examples of this phenomenon. For this study, we took advantage of the PRECISESADS IMI project, which gathered joint data from 2500 individuals with systemic autoimmune diseases, including systemic lupus erythematosus (SLE), systemic sclerosis (SSc), primary Sjögren’s syndrome (pSS), rheumatoid arthritis (RA), primary antiphospholipid syndrome (PAPS), and mixed connective tissue disease (MCTD), and aimed to determine such epigenetic modifications in SLE, SSc, pSS, and RA patients. Here, we performed a set of measures in several countries from the north and south of Europe. We observed that autoimmune patients from the north of Europe presented a higher hypomethylated profile associated with an increased gene expression than patients from the south. These genes were associated with interferon (IFN) pathways, immunity, and the pathways associated with cellular metabolism. Since the IFN scores were increased in this population, these patients presented a more inflammatory profile. To conclude, the geographical location of patients with autoimmune diseases has an impact on DNA methylation, RNA expression, and immunological profiles.


2021 ◽  
Vol 17 ◽  
Author(s):  
Jozélio Freire Carvalho ◽  
Roberto Paulo Correia de Araujo ◽  
Carlos Mauricio Cardeal Mendes ◽  
Thelma Larocca Skare

Aim: To study the clinical and laboratory findings between patients with primary Antiphospholipid Syndrome (pAPS) with and without LR. Background: Livedo Reticularis (LR) is a common manifestation of Antiphospholipid Syndrome (APS). Although no previous study evaluated patients with and without LR. Methods: A transversal study including 66 pAPS patients was performed. Demographical, anthropometric, medication use, antiphospholipid antibodies profile data were evaluated, and LR's clinical and laboratory features. Patients were subdivided into one of two groups: pAPS with LR and pAPS without LR. Results: Both groups were alike concerning demographics and anthropometrics. Interestingly, the frequency of stroke (28.5 vs. 7.5%, p=0.04), as well as of Sneddon’s syndrome (100 vs. 30.0%, p<0.0001), were higher in pAPS with LR than the other group. Conversely, patients in the pAPS without LR group had more thyroidopathy than those in the pAPS with LR group (80% vs. 50% %, p=0.03). Conclusion: Patients with pAPS and LR have more stroke and seem to be protected from thyroidopathy. Careful follow-up of these patients is therefore advised.


Lupus ◽  
2021 ◽  
pp. 096120332110380
Author(s):  
Carla Baleeiro Silva ◽  
Camila SC Duran ◽  
Flavio Signorelli ◽  
Gustavo GM Balbi ◽  
Eloisa Bonfá ◽  
...  

Background Characteristics of primary APS (PAPS) in the youth population have never been studied. In contrast with children, pregnancy is genuinely relevant in the youth age, and understanding clinical characteristics of PAPS patients within this specific age stratum may also provide insights regarding the well-known risk of poor obstetric outcomes during the adolescence. Objective To evaluate clinical and laboratory characteristics of patients with youth-onset PAPS (15–24 years) and compare them with adult-onset PAPS (over 24 years old). Methods This was a cross-sectional study derived from two rheumatology outpatient clinics. Patients who fulfilled Sidney criteria and who were 15 years of age or older at disease onset were included. Secondary APS patients were excluded. We subdivided patients into two groups: youth- (15–24 years) and adult-onset (over 24 years) and compared them regarding demographic characteristics, criteria and non-criteria manifestations, cardiovascular risk factors, and aPL status. For the pregnancy outcomes analysis, ever-pregnant patients were divided in three groups: youth-onset, early adult-onset (25–34 years), and late adult-onset (35–49 years). Results A total of 250 consecutive PAPS patients were included. Groups had a comparable female and Caucasian distribution. We found a similar disease duration (14.0±7.9 vs 17.0±10.1 years, p = 0.079) and similar rates of thrombotic arterial (34.2% vs. 42.0%, p = 0.250) and venous events (69.7% vs. 69.5%, p = 0.975) between them. Skin ulcers were more frequent in the youth-onset group (17.1% vs. 4.0%, p = 0.001), whereas nephropathy was less common (1.3% vs. 8.0%, p = 0.039). No differences were observed for the other criteria and non-criteria manifestations. The adult-onset group presented more frequently with hypertension ( p = 0.002), hyperlipidemia ( p = 0.008), and smoking ( p = 0.003). The youth-onset group presented a higher frequency of obstetric events as the first manifestation of PAPS (30.3% vs. 21.7%, p = 0.005), with worse pregnancy outcomes, namely, fetal death (58.5% vs. 46.4% vs. 24.1%, p = 0.012) and premature delivery (35.8% vs. 19.0% vs. 10.3%, p = 0.016). Of note, all groups had a comparable number of pregnancies (2.81±2.52 vs 2.74±2.07, p = 0.899). Conclusion This study provides novel evidence that youth-onset PAPS presents a higher frequency of obstetric complications as its first manifestation, with an increased risk of fetal death and preterm delivery. Early recognition of this condition by obstetricians is essential to improve prognosis.


CHEST Journal ◽  
2021 ◽  
Vol 160 (4) ◽  
pp. A2239
Author(s):  
Sharad Oli ◽  
Ankur Sinha ◽  
Resha Khanal ◽  
Varun tej Gonuguntla ◽  
Shital Oli ◽  
...  

CHEST Journal ◽  
2021 ◽  
Vol 160 (4) ◽  
pp. A2213-A2214
Author(s):  
Sharad Oli ◽  
Resha Khanal ◽  
Ankur Sinha ◽  
Varun tej Gonuguntla ◽  
kiran para ◽  
...  

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