Efficacy of plasma exchange therapy for diffuse alveolar hemorrhage in patients with microscopic polyangiitis

Abe, Yoshiyuki; Kusaoi, Makio; Tada, Kurisu; Yamaji, Katsuhiko; Tamura, Naoto

doi:10.1111/1744-9987.13824

Cited by 5 publications

(4 citation statements)

References 27 publications

(41 reference statements)

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“…Although methods of PEx as well as immunosuppression therapy, were not unified before starting treatment, the content was almost uniform as described, indicating that this study can offer relatively high-quality real-world data. Since refractory anti-MDA5 Ab-positive CADM with RP-ILD is a rare disease, it is necessary to establish the therapeutic evidence of PEx by accumulating cases at multiple institutions through the apheresis registry [33]. Therefore, although the result of this study is descriptive, we believe that PEx is a promising option to induce remission as a bridging therapy and to improve the outcome of refractory anti-MDA5 Abpositive CADM with RP-ILD though our real-world data.…”

Section: Discussionmentioning

confidence: 82%

Effectiveness and safety of plasma exchange for anti‐MDA5 antibody–positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series

Watanabe,

Taniguchi,

Ogura

et al. 2024

Ther Apher Dial

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IntroductionClinically 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.MethodsWe 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.ResultsFive 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.ConclusionPEx 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.

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Section: Discussionmentioning

confidence: 82%

Effectiveness and safety of plasma exchange for anti‐MDA5 antibody–positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series

Watanabe,

Taniguchi,

Ogura

et al. 2024

Ther Apher Dial

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“…However, in a smaller study [19] plasma exchange showed potential survival benefit at 1 year in patients with MPA and diffuse alveolar hemorrhage. This was in only 4 patients who were compared to 16 historical control patients, so the data need to be interpreted as preliminary and maybe suggesting a potential use in some specific patient groups.…”

Section: Key Pointsmentioning

confidence: 85%

Current treatment approach to ANCA-associated vasculitis

Yazici

2023

Current Opinion in Rheumatology

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Purpose of review This review will attempt to summarize the most potentially impactful new data on the way ANCA-associated vasculitis (AAV) is diagnosed, treated, and monitored. Recent findings The newly developed classification criteria for AAV have serious methodological issues that need to be addressed before they are widely adopted. The newly approved drugs and studies into both achieving remission and maintaining it have added to our overall knowledge of managing AAV and should hopefully contribute to improving outcomes in AAV. Summary The diagnosis, treatment and monitoring of AAV have seen major improvements in the last two years. The remaining issues outlined in this review still need to be addressed to best serve AAV patients.

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“…With the emergence of LPHS cases throughout the country, many centres in Sri Lanka use PLEX as a therapeutic measure. Evidence for PLEX was based on the benefit observed in the treatment of pulmonary haemorrhage in autoimmune diseases such as Goodpasture's syndrome, ANCA-associated vasculitis, systemic lupus erythematosus (SLE), and other autoimmune reno-pulmonary syndromes [23][24][25]. The above diseases arise due to either direct deposition of autoantibodies or antigen-antibody complexes in the alveolar-capillary membrane, resulting in inflammation, pulmonary vasculitis, tissue damage, necrosis, and pulmonary haemorrhage.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics and outcomes of pulmonary haemorrhage in leptospirosis: A retrospective cohort study from Sri Lanka

Ruwanpathirana,

Perera,

Rambukwella

et al. 2024

Preprint

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Background Leptospirosis, a tropical, spirochaetal infection presents as an acute febrile illness with organ injury. There is a paucity of data on clinical characteristics and treatment for Leptospirosis Pulmonary Haemorrhage Syndrome (LPHS). Methodology and principal findings A retrospective cohort study was conducted including all patients with LPHS treated in the medical Intensive Care Unit (ICU), National Hospital Sri Lanka from 2019 to 2022 to describe the clinical characteristics and factors related to poor outcomes. Survival of patients who received different treatment modalities for LPHS was compared. Seventy patients were studied with a mean age of 42.69 ± 27.84 years and 61 (87.1%) males. Forty-nine (70%) were mechanically ventilated and 16 (22.9%) died. Higher heart rate, higher lactate, lower pH on admission to ICU, and requirement of blood product transfusion were associated with increased mortality. Patients were treated with plasmapheresis (PLEX), intranasal desmopressin (DDAVP), and intravenous steroids alone or in combination as the specific treatment for LPHS. Seven (10%) patients were treated with PLEX alone, 13 (18.6%) with PLEX + DDAVP, 46 (65.7%) with PLEX + DDAVP + steroids, and 4(5.7%) were treated with steroids alone. All patients who received PLEX and DDAVP combination survived. There were 11 (23.9%) deaths in the PLEX+ DDAVP + steroids group, 3 (49.2%) in the PLEX alone group, and 2 (50%) in the steroids alone group. Mortality was least when PLEX was done for 3 days. Twenty-five (35.7%) patients developed hospital-acquired infections and risk factors were mechanical ventilation and longer ICU stay. Conclusions Presence of tachycardia, acidosis, and high lactate predicted death in LPHS. PLEX and DDAVP combination had better survival than other treatments alone or in combination for LPHS. Randomized clinical trials are urgently needed to identify the role of PLEX and DDAVP in treating LPHS.

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Efficacy of plasma exchange therapy for diffuse alveolar hemorrhage in patients with microscopic polyangiitis

Cited by 5 publications

References 27 publications

Effectiveness and safety of plasma exchange for anti‐MDA5 antibody–positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series

Effectiveness and safety of plasma exchange for anti‐MDA5 antibody–positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series

Current treatment approach to ANCA-associated vasculitis

Clinical characteristics and outcomes of pulmonary haemorrhage in leptospirosis: A retrospective cohort study from Sri Lanka

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