A Case of Clinically Amyopathic Dermatomyositis that was Refractory to Intensive Immunosuppressive Therapy including Tofacitinib, but Successfully Treated with Plasma Exchange Therapy

ABSTRACT Clinically amyopathic dermatomyositis (CADM) patients often develop rapidly progressive interstitial lung disease (RP-ILD). A high level of anti-melanoma differentiation-associated gene 5 antibodies (anti-MDA5 Ab) before treatment is associated with RP-ILD development, a poor treatment response, and poor survival. The prognosis of CADM patients remains poor due to ILD even with combined intensive immunosuppressive therapy. Recently, several additional therapies, including tofacitinib (TOF) and plasma exchange (PE) therapy, have been reported to be effective. We herein report a case of CADM-ILD with a high level of anti-MDA5 Ab that was refractory to combined intensive immunosuppressive therapy including TOF, but successfully treated with PE. The following are possible reasons why TOF was ineffective: 1) cytokines that were not suppressed by TOF played an important role in RP-ILD; 2) TOF was administered later than previously reported; and 3) TOF did not suppress pathological substances such as antibodies. On the other hand, PE removes cytokines and various pathological substances. Therefore, PE may be a more reasonable additional therapy for intractable CADM-ILD.

A case study of feline triaditis

Triaditis is an inflammatory condition involving three specific organs, namely the pancreas, liver, and intestines. The most common clinical signs of triaditis are vomiting, diarrhea, jaundice, and loss of appetite. Therefore, this case study was con-ducted to determine cases of triaditis in cats with these clinical signs. This case study was carried out on five cats with diar-rhea, lethargy, and loss of appetite. The fecal scores of cats with diarrhea were 2 and 3. Based on physical examination, there were two cats with hepatomegaly and three cats with jaundice on their mucosa. Treatments for five cats with diarrhea were antibiotic combination (sulfadiazine-trimetropine 50 mg/kg BW) and combination of antispasmodic, analgesic, and antipyret-ic (dipyrone-lidocaine 5 mg/kg BW). Treatments for three cats with jaundice were asering fluid therapy and oral medication (ursodeoxycholic acid 8 mg/kg BW). Anthelmintic combination (fenbendazole, praziquantel, and pyrantel pamoate 0.07 g/kg BW) was given as an additional therapy for cats with intestinal worms. The two cats with diarrhea, hepatomegaly and jaun-dice were not survive after about one week of treatment, while the other one can survive until now

Treatment Considerations for Obstructive Sleep Apnea in Pediatric Down Syndrome

Children with Down syndrome (DS) are at high risk for developing obstructive sleep apnea (OSA) compared to children without DS. The negative impact of OSA on health, behavior, and cognitive development in children with DS highlights the importance of timely and effective treatment. Due to the higher prevalence of craniofacial and airway abnormalities, obesity, and hypotonia in patients with DS, residual OSA can still occur after exhausting first-line options. While treatment commonly includes adenotonsillectomy (AT) and continuous positive airway pressure (CPAP) therapy, additional therapy such as medical management and/or adjuvant surgical procedures need to be considered in refractory OSA. Given the significant comorbidities secondary to untreated OSA in children with DS, such as cardiovascular and neurobehavioral consequences, more robust randomized trials in this patient population are needed to produce treatment guidelines separate from those for the general pediatric population of otherwise healthy children with OSA. Further studies are also needed to look at desensitization and optimization of CPAP use in patients with DS and OSA.

Assessing Clinical Outcomes in Patients Undergoing Splenectomy for Immune Thrombocytopenia: A Real World Perspective

Background: Immune thrombocytopenia (ITP) is an acquired immune-mediated disorder characterized by frequent relapses. Splenectomy was considered an integral part of ITP therapy until the inception of newer agents, namely rituximab and thrombopoietin receptor agonists (TPO-RA) over the last 20 years. Recently the role and timing of splenectomy have become an area of debate. Studies regarding optimal predictors of response have not demonstrated reliable clinical or laboratory factors. We chose to evaluate the most recent practices and outcomes at Montefiore Medical Center to assess the role of splenectomy in ITP treatment in our diverse, real world patient population. Methods: We conducted a retrospective cohort study of patients who had undergone splenectomy for ITP at Montefiore between January 1, 2015 and July 1, 2021. Collected data included demographics, comorbidities, prior lines of treatment, and splenectomy outcomes. Outcome data included mortality and need for post-splenectomy treatment. Response rates were assessed by American Society of Hematology (ASH) guidelines. Results: The study population comprised 29 patients who underwent splenectomy for ITP as primary indication. Patients were 59% (N=17) female, with a median age of 45 years. Our multi-ethnic cohort included 11 (40%) Hispanic, 7 (24%) African American, and 6 (21%) Caucasian patients. Five patients chose not to specify. Significant comorbidities prior to undergoing surgery were present in 79% (N=23) of patients, with the average number being two. Of these, 41% (N=12) had a comorbid autoimmune condition. Two were HIV positive. Initial treatment consisted of steroids, IVIG (or WinRho), or both for all patients. Only four patients failed to respond to these therapies; one of whom experienced remission following splenectomy and one of whom expired within 30 days of surgery. Fourteen patients (48%) continued to receive repeat doses of steroids and/or IVIG until surgery. Time to splenectomy was variable, range 0-9 years. Additional therapy following relapse on initial treatment consisted of TPO-RA (11/29, 38%); rituximab (11/29, 38%); immunosuppressive therapy (1/29, 3%); and danazol (3/29, 10%). Response rates for TPO-RA (8/11, 73%), danazol (2/3, 67%), and immunosuppressive therapy (1/1) were greater than that for rituximab infusion (4/11, 36%). Overall, the average number of unique lines of therapy was 2.8 (median=2) and average years from diagnosis to splenectomy was 2.8 (median=1, range 0-11). At a median time from splenectomy to last follow up (or time of death) of 971 days (median=876 days), 18 patients (62.1%) had remained off medical therapy with complete response (CR) following splenectomy. Three patients (10.3%) transiently required additional therapy but subsequently achieved CR and remained off medical therapy for greater than a year. Six patients (20.7%) continued to rely on medical therapy. Two patients (6.9%) died within 30 days of splenectomy; one of unknown causes and one of pulmonary embolism (despite thromboprophylaxis) in the setting of TPO-RA therapy. There was no difference in continued reliance on medical therapy in patients who underwent splenectomy earlier versus later in their ITP course. No differences in outcomes were noted in patients with underlying comorbidities. No differences in outcome were noted between Hispanic, African American, and Caucasian patients. Conclusion: Our review of ITP splenectomies in a real world patient population revealed significant differences in treatment practices and timing of surgical intervention. We did not note differences in splenectomy outcomes across patients of different backgrounds or with comorbidities, confirming that these should not play a role in treatment decisions. Patients who failed to respond to steroids and/or IVIG had lower response rates to splenectomy as has been previously hypothesized, though these represented a very small subset of our study population. Further evaluation of compliance, tolerance, and social factors (i.e. insurance, etc) may assist with understanding differences in response rates to various second line medical therapies. Splenectomy continues to offer high rates of sustained CR for a variety of patients, though reliable predictors of response remain elusive and should continue to be an area of active research. Disclosures No relevant conflicts of interest to declare.

Psoriasis Therapy Beyond Biologics

Although psoriasis patients have benefited from the advent of biologic treatments over the past two decades, these medications are not appropriate for all patients and can be augmented by additional therapy. Differences among the manifold options can be difficult to parse, though essential for matching treatment with an individual patient. UV-light therapies, including both UV-B and psoralen with UV-A light, continue to play an important role in treatment, as do non-biologic systemic options including methotrexate, cyclosporine, apremilast, and acitretin. Recent years have seen a dramatic expansion in available topical therapies, the most common modality for the treatment of psoriasis, including new foam, spray, lotion, and cream formulations of topical corticosteroids (TCS) and new fixed-dose combination offerings of TCS with tazarotene and calcipotriene. Newer advances, including the oral tyrosine kinase 2 inhibitor deucravacitinib and non-steroidal topicals such as roflumilast, a PDE-4 inhibitor, and tapinarof, a first-in-class non-steroidal small-molecule, will soon provide even more options for treatment. It is vital for clinicians to remain aware of this ever-expanding armamentarium, allowing for more productive shared decision-making with patients, improved satisfaction, and better disease control.

Adjunctive therapy for acute musculoskeletal pain

Acute musculoskeletal pain (MSP) is one of the leading complaints at patients' admission. Acute MSP is usually localized in the lower back, neck, shoulder girdles, and shins. With an increase in the number of obese and hypodynamic people, the prevalence of MSP increases, especially lower back pain. Treatment of acute MSP in the back consists of informing the patient about a favorable prognosis of the disease, recommendations to maintain daily activity, primary and additional pharmacotherapy. Primary or main pharmacotherapy of acute MSP in the back includes non-steroidal anti-inflammatory drugs (NSAIDs). Adjunctive therapy is usually used in combination with NSAIDs and is aimed to increase the effectiveness of treatment and reduce the duration of NSAIDs use. As an additional therapy, muscle relaxants or B vitamins can be prescribed. The efficacy and safety of combination therapy of NSAIDs and high-dose complexes of B vitamins have been demonstrated in clinical trials involving patients with acute back pain.

Yoga as adjunctive therapy in the treatment of people with anorexia nervosa: a Delphi study

Background There is preliminary evidence to suggest that yoga can be beneficial in reducing anxiety, depression and general eating disorder symptoms in people with Anorexia Nervosa (AN). It is unclear whether the therapeutic benefits of yoga are supported or utilised in the treatment of AN amongst clinical experts. The present study aimed to explore and synthesise expert opinion on the use of yoga as an adjunctive therapy in the management of anxiety, depression and over-exercise in individuals with AN. Methods A Delphi methodology was employed, with clinicians considered experts in the treatment of AN recruited internationally to form the panel (n = 18). The first iteration of questionnaires comprised of four open-ended questions concerning the experts’ understanding of the term yoga and opinions on its’ use in therapy generally and more specifically in the treatment of AN. Using content analysis, statements were derived from this data and included as Likert-based items in two subsequent rounds where panellists rated their level of agreement on each item. Seventeen out of 18 respondents completed all three iterations. Results Consensus (level of agreement defined at ≥ 85%) was achieved for 36.47% of the items included in the second and third rounds. The panel reached consensus on items defining yoga and pertaining to its’ general benefits. The panel agreed that yoga is a adjunct therapy for various problems, consensus was not achieved on the specific use of yoga as an adjunct therapy in the treatment of comorbid anxiety, depression or trauma in patients with AN. Although the expert panel acknowledged a number of benefits for use of yoga in AN, they strongly endorsed that future research should evaluate the potential risks of using yoga as an embodied practice. Conclusions It is possible that yoga could be considered for inclusion in future guidelines if supported by empirical research. We conclude that there seems to be enough consensus that such further scientific investigation is warranted. Plain English summary This study aimed to explore expert opinion on the use of yoga as an adjunctive therapy in the management of anxiety, depression and over-exercise in individuals with Anorexia Nervosa (AN). Clinicians considered experts in the treatment of AN recruited internationally to form the panel (n = 18). Experts were asked about their understanding of the term yoga and their opinions on its’ use in therapy. The panel reached consensus on items defining yoga and pertaining to its’ general benefits. Although the panel agreed that yoga is a nice additional therapy for various problems, consensus was not achieved on the use of yoga as an additional therapy in the treatment of specific problems like anxiety, depression or trauma in people with AN. The expert panel acknowledged a number of benefits for use of yoga in AN. However the panel strongly considered that future research should evaluate the potential risks of using yoga as an embodied practice. The areas of collective agreement gained in the study can serve as preliminary guidelines for the use of yoga in AN whilst guiding future research directions.

333 THE LONG-TERM TREATMENT OUTCOMES OF ESOPHAGEAL SCC WITH MM/SM1 INVASION AFTER ENDOSCOPIC RESECTION

Resent advances in endoscopic resection (ER) provide us increasing chances for resecting esophageal SCC with muscularis mucosae (MM) and submucosal (SM) invasion. We perform additional therapy such as chemoradiotherapy (CRT) or esophagectomy considering the risk of metastasis and patient’s condition. However, there is only a few reports about long-term outcome after ER for such cases. Methods We retrospectively studied 188 case of esophageal SCC with pathological MM invasion resected by ER (161 cases) and SM1(27 cases) from 2005 to 2016 in Cancer Institute Hospital. We recommended CRT or esophagectomy as additional treatment for the cases which had lymphovascular invasion (LVI) or DI (droplet infiltration) considering patients’ conditions. Median observation period of them were 71 months. Results The SM1 cases had significantly higher rate of LVI than the MM cases. Of 161 MM cases, 2 cases had recurrence, however, they are alive after CRT or CRT following esophagectomy. Of 27 SM1 cases, 3 cases had LN recurrence, underwent RT or CRT or CRT following esophagectomy, one case died of esophageal SCC and the others survive. The rate of metastatic cases was higher in SM1 than that in MM. Overall survival rate in 5 years were 89.8% in MM and 81.8% in SM1. Cause specific survival rate in 5 years were 100% in both MM and SM1. Conclusion The long-term outcomes of ER for MM/SM1 invasive esophageal SCC was good with appropriate additional therapy which we should perform in high risk cases for LN metastasis considering patients’ condition.