ABSTRACT
INTRODUCTION: Clinically amyopathic dermatomyositis (CADM) with anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab) with rapidly progressive interstitial lung disease (RP-ILD) is often refractory for intensive immunosuppression. In this study, we verified the effectiveness and safety of plasma exchange (PEx) for this lethal disease. METHODS: We retrospectively examined the clinical course and adverse effect (AE) of 12 patients with anti-MDA5 Ab-positive CADM between January 2017 and December 2021 in our hospital. RESULTS: Five out of six patients treated with simple PEx using fresh frozen plasma or 5% albumin survived with or without home oxygen therapy. Multiple PEx (15-20 times) were required to achieve satisfactory improvement as well as remission of CADM. The AEs caused by PEx were resolved using conventional methods. CONCLUSION: PEx might be a promising option for controlling the disease activity of anti-MDA5 Ab-positive CADM with severe RP-ILD and may contribute to better survival.
Subject(s)
Dermatomyositis , Interferon-Induced Helicase, IFIH1 , Lung Diseases, Interstitial , Plasma Exchange , Humans , Lung Diseases, Interstitial/therapy , Lung Diseases, Interstitial/immunology , Dermatomyositis/immunology , Dermatomyositis/therapy , Dermatomyositis/complications , Plasma Exchange/methods , Male , Female , Middle Aged , Interferon-Induced Helicase, IFIH1/immunology , Retrospective Studies , Adult , Aged , Treatment Outcome , Disease Progression , Autoantibodies/bloodABSTRACT
Omental wrapping is a common cause of peritoneal catheter malfunction. This diagnosis should be confirmed by radiography before proceeding with surgical omentectomy. We report two cases of peritoneal dialysis (PD) catheter outflow obstruction from our developing PD program in Grenada, in which contrast studies accurately diagnosed omental wrap, allowing for prompt surgical correction. In both cases, the contrast study indicated the presence of omental wrapping, confirmed at time of surgical correction. Radiographic features of omental wrap are distinctive, which allows for reliable differentiation from other causes of obstruction. Radiographic contrast study reliably diagnoses the cause of peritoneal catheter obstruction, permitting prompt diagnosis and treatment. This is vital for regions with limited access to haemodialysis.
