Steroid-Sparing Effect, Effectiveness, and Tolerability of Mepolizumab with EGPA in Real World Clinical Practice

BackgroundEosinophilic polyangiitisgranulomatosa (EGPA) is a vasculitis syndrome that affects small and medium-sized blood vessels throughout the body. The mainstay of treatment is steroids, but there are no standardized doses or durations for steroids in combination with mepolizumab. MethodsWe investigated the efficacy, steroid administration, and course of steroid reduction in five patients who were diagnosed with EGPA and treated with mepolizumab for 1 year. Five patients who were diagnosed with EGPA using the American College of Rheumatology criteria and treated with mepolizumab for 1 year were included in our study from 2018–2020.ResultsEosinophil levels as well as the Birmingham Vasculitis Activity Score (BVAS), remission rate, annual relapse rate, and steroid dosage were observed at 1 year after mepolizumab treatment. Fifty-two weeks after mepolizumab treatment, eosinophils, BVAS, and the steroid dosage showed a trend toward improvement in four out of five patients. In the one patient who received no dose reduction, there was no exacerbation of bronchial asthma that had previously occurred during the follow-up period. Although neurological symptoms often remain even in remission, all patients with neurological symptoms at diagnosis improved. There were no cases of relapse in this study, including two anti-neutrophil cytoplasmic antibody-positive cases. ConclusionsManagement that includes early administration of mepolizumab may improve patients’ quality of life by alleviating lingering neurological symptoms that are caused by EGPA.

Effects of Allogeneic Mesenchymal Stem Cell Transplantation in Dogs with Inflammatory Bowel Disease Treated with and without Corticosteroids

Mesenchymal stem cells have proven to be a promising alternative to conventional steroids to treat canine inflammatory bowel disease (IBD). However, their administration requires a washout period of immunosuppressive drugs that can lead to an exacerbation of the symptoms. Therefore, the feasibility and effects of the combined application of stem cells and prednisone in IBD-dogs without adequate response to corticosteroids was evaluated for the first time in this study over a long- term follow up. Two groups of dogs with IBD, one without treatment and another with prednisone treatment, received a single infusion of stem cells. The clinical indices, albumin and cobalamin were determined prior to the infusion and after one, three, six and 12 months. In both groups, all parameters significantly improved at each time point. In parallel, the steroid dosage was gradually reduced until it was suppressed in all patients a year after the cell therapy. Therefore, cell therapy can significantly and safely improve the disease condition in dogs with IBD receiving or not receiving prednisone. Furthermore, the steroid dosage can be significantly reduced or cancelled after the stem cell infusion. Their beneficial effects are stable over time and are long lasting.

Dupilumab: a new contestant to corticosteroid in allergic bronchopulmonary aspergillosis

ABSTRACT Allergic bronchopulmonary aspergillosis (ABPA) is a disease characterized by severe disability with recurrent wheezing and shortness of breath. The current recommended therapy is daily oral corticosteroids +/− oral antifungal therapy. Despite this, many patients continue to have severe symptoms, and others require fairly high daily oral corticosteroid dosing to achieve control, which in turn may induce the well-known effects of long term steroid use. The anti-interleukin drugs have been reported to help improve daily symptoms and reduce steroid requirements. Much of the literature highlights the benefit of omalizumab. We present a case of dupilumab as add-on therapy in a patient with ABPA, which allowed us to reduce daily steroid dosage.

Baricitinib in the treatment of rheumatoid arthritis: clinical and ultrasound evaluation of a real-life single-centre experience

Background: Ultrasound (US) is useful in monitoring RA patients, with the US7 score allowing grey-scale and power-Doppler (PD) semi-quantitative evaluation of synovitis and teno-synovitis. We evaluated real-life efficacy and safety of Baricitinib, an oral selective JAK1-2 inhibitor, in RA patients using clinical, clinimetric, and US assessments. Methods: Disease activity score in 28 joints calculated with C-reactive protein (DAS28-CRP), disease activity score in 28 joints calculated with erythrocyte sedimentation rate (DAS28-ESR), clinical disease activity index (CDAI), simplified disease activity index (SDAI), visual analogue scale (VAS)-pain, health assessment questionnaire (HAQ), COCHIN scale, adverse events (AE), concomitant medications, laboratory parameters, and US7 were performed/recorded at baseline, 1, 3, and 6 months in RA patients starting Baricitinib. Responder/non-responder status was determined according to the EULAR Response Criteria at 3 months. SDAI clinical remission or low disease activity (LDA) were calculated at 3 and 6 months. Results: In 43 enrolled patients, a significant improvement in disease activity and US7 components (except tendon PD) and a reduction of steroid dosage were observed. Responders at 3 months showed a significantly higher reduction of CDAI, SDAI, COCHIN scale, VAS-pain, and US7 synovialPD, compared with non-responders. At 3 and 6 months, remission/LDA was achieved by 12.8/53.8% and 21.6/51.3% patients, respectively. The csDMARD co-treatment was independently associated with remission/LDA at 3 months. Safety-related drop-outs were in line with literature data. The steroid dosage was associated with AE development at 6 months. Conclusion: The real-life data, also obtained with US evaluation, confirmed the Baricitinib efficacy in RA disease control, as well as the utility of assessment during the follow up of disease activity.

Membranous nephropathy associated with thrombospondin type-1 domain-containing 7A (THSD7A) in an adult woman with eosinophilia

A 30-year-old woman on steroid therapy for eosinophilia presented with nephrotic syndrome during steroid tapering. She was diagnosed with membranous nephropathy (MN) stage II–III (positive for IgG1 and IgG4) by renal biopsy. There was no evidence of secondary MN. Her urinary protein level was controlled to 0.5 g/day or less, and her eosinophil count in white blood cell differential was stabilized at less than 10% without increasing the steroid dosage. The renal specimen did not show any enhanced granular expression of PLA2R along the glomerular basement membrane, and PLA2R was not detected in the patient’s serum. On retrospective analysis, enhanced granular staining for thrombospondin type-1 domain-containing 7A (THSD7A) in the glomeruli was detected in the biopsy, and anti-THSD7A IgG was detected in the serum using a commercial indirect immunofluorescence test (IFT). Based on these, the case was considered as THSD7A-associated MN with comorbid eosinophilia. The causal relationship between THSD7A-related MN and eosinophilia was unclear. However, a few cases of THSD7A-associated MN with eosinophilia have been reported, and further clarification on the relationship between THSD7A-related MN and eosinophilia is warranted.