scholarly journals Treatment of MDA5-positive dermatomyositis complicated by gangrenous cholecystitis with tofacitinib

Author(s):  
Man Luo ◽  
Long Chen ◽  
Huan He ◽  
Fang He

Abstract BackgroudDermatomyositis is a diffuse skeletal myositis disease with a variety of causes.The onset is mainly related to humoral immune abnormalities.Some therapeutic drugs,such as hormones and immunosu -ppressants,have poor therapeutic effects.In recent years, tofacitinib has been reported to be effective in the treatment of dermatomyositis.But there are many reports about the treatment of autoimmune diseases and infectious diseases with tofacitinib.Case presentationWe report a case of MDA5 antibody-positive dermatomyositis that was relieved after treatment with tofacitinib,during which gallbladder gangrene and suppurative cholecystitis occurred.After cholecystectomy,we continued to use tofacitinib and achieved good therapeutic effect. ConclusionsTofacitinib is effective in the treatment of MDA5 antibody-positive dermatomyositis,but the risk of infection is increased.It can still be used after infection control.A close follow-up should be performed during the use of tofacitinib.

1994 ◽  
Vol 30 (2) ◽  
pp. 253 ◽  
Author(s):  
Seog Hee Park ◽  
Kyung Sub Shinn ◽  
Ki Tae Kim ◽  
Seong Tae Hahn ◽  
Choon Yul Kim ◽  
...  

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Fajer A Altamimi ◽  
Una Martin

Abstract Background/Aims  Telemedicine can be broadly defined as the use of telecommunication technologies to provide medical information and services. It can be audio, visual, or text. Its use has increased dramatically during the COVID-19 pandemic to ensure patient and healthcare worker safety. Any healthcare professional can engage with it. It carries benefits like reduced stress and expense of traveling, maintenance of social distancing, and reduced risk of infection. There are some potential drawbacks such as lack of physical examination, liability and technological issues. Methods  A questionnaire was sent to 200 patients, selected from different virtual clinics (new and review, doctor and ANP led) run between March and May 2020 in the rheumatology department of University Hospital Waterford. We formulated 14 questions to cover the following aspects: demography, the purpose of the consult, punctuality, feedback, medico-legal concerns, and free text for comments. A self-addressed return envelope was included. Results  83 responses were received. 2 were excluded. The ratio of females to male respondents was 59: 41, with the majority over 60 years old. The main appointment type was review 67 (83%). 80% of patients were called either before or at the time of their scheduled appointment. The vast majority (98.8%) of our patients had confidence in our data protection and trusted our system to maintain their confidentiality. 95% stated that they felt comfortable, were given enough time to explain their health problem and felt free from stress. The respondents who preferred attending the clinic in person (17 in total) compared to the virtual were mostly follow up patients- 12 vs. 5 new. Conclusion  Patient satisfaction among those surveyed was high, despite having to introduce the service abruptly during the COVID-19 pandemic. There are many improvements we can adopt to improve our service and even maintain after the pandemic as a way of communicating with our stable patients. As we are covering a large geographical catchment, we can continue to implement the virtual clinic for some appointments. We should prioritize our efforts on identifying the right patient and the type of service we can offer, further training of staff, and increasing awareness of the patients as to how to get the most out of a virtual appointment. Disclosure  F.A. Altamimi: None. U. Martin: None. C. Sheehy: None.


2019 ◽  
Vol 25 (1) ◽  
Author(s):  
Danielle Whiting ◽  
Ian Rudd ◽  
Amit Goel ◽  
Seshadri Sriprasad ◽  
Sanjeev Madaan

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.


2020 ◽  
pp. 1-4
Author(s):  
Valentina Orlando ◽  
Pietro Spennato ◽  
Maria De Liso ◽  
Vincenzo Trischitta ◽  
Alessia Imperato ◽  
...  

<b><i>Introduction:</i></b> Hydrocephalus is not usually part of Down syndrome (DS). Fourth ventricle outlet obstruction is a rare cause of obstructive hydrocephalus, difficult to diagnose, because tetraventricular dilatation may suggest a communicant/nonobstructive hydrocephalus. <b><i>Case Presentation:</i></b> We describe the case of a 6-year-old boy with obstructive tetraventricular hydrocephalus, caused by Luschka and Magen­die foramina obstruction and diverticular enlargement of Luschka foramina (the so-called fourth ventricle outlet obstruction) associated with DS. He was treated with endoscopic third ventriculostomy (ETV) without complications, and a follow-up MRI revealed reduction of the ventricles, disappearance of the diverticula, and patency of the ventriculostomy. <b><i>Conclusion:</i></b> Diverticular enlargement of Luschka foramina is an important radiological finding for obstructive tetraventricular hydrocephalus. ETV is a viable option in tetraventricular obstructive hydrocephalus in DS.


2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Jai Kumar ◽  
Mohammad Irfaan Albeerdy ◽  
Nadeem Ahmed Shaikh ◽  
Abdul Hafeez Qureshi

Abstract Background Peutz-Jeghers syndrome is an autosomal dominant disease characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract (GIT). There have also been cases of extra GIT polyps such as the renal pelvis, urinary bladder, lungs and nares. Bladder hamartoma is an extremely rare finding, with only 12 cases described in the literature up to now. The rarity of the condition necessitates a comprehensive compilation of managements up to now so as to provide a better tool for the treatment of such conditions in the future. Case presentation A twenty-year-old male, known to have Peutz-Jeghers syndrome, presented to us complaining of obstructive urinary symptoms. A urethrogram done showed a filling defect at the base of the urinary bladder. The mass was resected transurethrally, and histopathology revealed a hamartoma of the bladder. The patient has since remained tumor-free on follow-up. Conclusions Transurethral resection of the bladder mass proved to be an effective therapy in this patient with no recurrence on the patient’s follow-up till now. There is still, however, a dearth of knowledge regarding the management of bladder hamartomas owing to the extreme rarity of the case.


2021 ◽  
Vol 16 (1) ◽  
pp. 395-398
Author(s):  
Yixiao Fu ◽  
Cuiping Zheng ◽  
Jian Huang ◽  
Shenghao Wu ◽  
Yanyan Dai

Abstract Background Duodenal adenocarcinoma (DA) with skin metastasis as initial manifestation is clinically rare. In this study, we report a rare case of skin metastasis of DA. Case presentation An 84-year-old male patient developed multiple ecchymoses on the trunk and lower extremities. Physical examination showed that the ecchymosis was dark red and had a hard texture, but showed no bulging, rupture, or tenderness. The skin biopsy implied skin metastatic adenocarcinoma. After an endoscopic duodenal biopsy, the patient was finally diagnosed with DA with skin metastasis. The patient received two courses of oral treatment of Tegafur (40 mg, bid d1–d14). However, the patient stopped taking Tegafur because of its poor effect and received Chinese medicine as a replacement treatment. Unfortunately, he was lost to follow-up. Conclusions Early diagnosis of DA metastasis is of significant importance as prognosis of these patients is poor.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Zai-Qiang Zhang ◽  
Jia-Wang Ding

Abstract Background While the perforation of the atrial wall and aortic sinus after closure of an atrial septal defect (ASD) is rare, it’s life-threatening, with rapid progress and high mortality. To the best of our knowledge, 21 similar cases have been reported since 1976. Case presentation We report a 16-year-old male whose atrial septal defect (ASD) was closed using a 12-mm Amplatzer septal occluder (ASO). Atrial wall and aortic sinus perforation occurred 3 months after transcatheter closure, and the patient was discharged after emergency operation. He was discharged on the 12th postoperative day in good overall condition. Conclusions With this case report, we want to illustrate that although percutaneous closure of ASD is regarded as a routine procedure, we should not forget the potentially lethal complications, especially cardiac erosion. Therefore, we should carefully evaluate the risk of erosion before surgery, and careful lifelong follow-up is needed.


2020 ◽  
Vol 26 (1) ◽  
Author(s):  
Nabil Nabil Moohialdin ◽  
Ahmad Shamsodini ◽  
Steven K. Wilson ◽  
Osama Abdeljaleel ◽  
Ibrahim Alnadhari ◽  
...  

Abstract Background Infection after the penile prosthesis can be devastating to both the patient and surgeon with various complications and consequences. After introduction of antibiotic-coated implants, the rate of infection has dramatically decreased, but still we see uncommon organisms causing infection. We present a first case report of penile prosthesis infection by brucellosis due to raw milk ingestion. To our knowledge, this is the first reported case of brucellosis penile prosthesis infection. Case presentation We present a first case report of penile prosthesis infection by brucellosis due to raw milk ingestion. A 75-year-old, diabetic male patient presented with penile prosthesis infection 5 months post-penile exchange surgery due to mechanical malfunctioning of 2-piece penile prosthesis which was inserted 11 years ago. The initial treatment with broad spectrum antibiotics did not subside the infection. After diagnosis of brucellosis, the antibiotic was changed to anti-brucellosis (Rifampicin + Tetracycline). The patient improved dramatically and was discharged home with smooth follow-up course. Conclusion Brucellosis can cause infection of penile prosthesis and can be treated with anti-brucellosis antibiotics without necessitating surgical intervention and removal of prosthesis.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Hiroshi Saito ◽  
Koichiro Sawada ◽  
Jyunichi Ogawa ◽  
Masashi Hashimoto ◽  
Masahiro Oshima ◽  
...  

Abstract Background Median arcuate ligament syndrome (MALS), which results from compression of the median arcuate ligament (MAL), is a rare cause of abdominal pain and weight loss. Treatment is dissection of the MAL; however, the laparoscopic procedure is not yet established and it involves the risk of major vascular injury, especially in cases with an anomaly. Case presentation A 47-year-old man was evaluated at the hospital for epigastric pain. Contrast computed tomography scan revealed stenosis of the celiac artery origin due to the MAL. An Adachi V type vascular anomaly was also observed. Laparoscopic treatment was performed to release pressure on the celiac artery. Laparoscopic ultrasonography was used to less invasively confirm the release of the MAL. Despite a concomitant Adachi V type vascular anomaly, surgery was safely performed using the laparoscopic magnification view and intraoperative ultrasonography. Follow-up ultrasonography confirmed the celiac artery stenosis has not recurred. Conclusions A rare case of MALS with an Adachi V type vascular anomaly is presented and the laparoscopic treatment is detailed.


Reports ◽  
2021 ◽  
Vol 4 (1) ◽  
pp. 1
Author(s):  
Juan Luis Fernández-Morera ◽  
Alfredo Renilla González ◽  
Carmen Elena Calvo Rodríguez ◽  
Judit Romano-García

Background: CTLA-4 and PD-1L are novel immune checkpoint targets for cancer treatment with specific side effects such as autoimmune diseases. Less frequently, the presence of several autoimmune diseases in the same patient has been described. In this communication, we illustrate the case of a 45-year-old patient with a previous diagnosis of advanced cancer that, after starting treatment with this immunotherapy, developed in the following months autoimmune diabetes, lymphocytic hypophysitis, and a Hashimoto thyroiditis in an abrupt and intense manner that would correspond to an autoimmune polyglandular disease. Discussion: The activation of autoimmunity and associated diseases is increasing in parallel with augmented indication of these immunotherapeutic treatments in cancer patients. A closer follow-up of these patients could be necessary for an optimal approach to this type of pathology. Conclusions: Different autoimmune diseases can converge in the same patient when immunotherapy for cancer is indicated to boost immune response against tumor, caused by altering immune tolerance.


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