Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. The management of PM-/DM-/CADM-associated ILD (PM/DM/CADM-ILD) requires careful evaluation of the disease severity and clinical subtype, including the ILD forms (acute/subacute or chronic), because of the substantial heterogeneity of their clinical courses. Recent studies have highlighted the importance of myositis-specific autoantibodies’ status, especially anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl tRNA synthetase (ARS) antibodies, in order to evaluate the clinical phenotypes and treatment of choice for PM/DM/CADM-ILD. Because the presence of the anti-MDA5 antibody is a strong predictor of a worse prognosis, combination treatment with glucocorticoids (GCs) and calcineurin inhibitors (CNIs; tacrolimus (TAC) or cyclosporin A (CsA)) is recommended for patients with anti-MDA5 antibody-positive DM/CADM-ILD. Rapidly progressive DM/CADM-ILD with the anti-MDA5 antibody is the most intractable condition, which requires immediate combined immunosuppressive therapy with GCs, CNIs, and intravenous cyclophosphamide. Additional salvage therapies (rituximab, tofacitinib, and plasma exchange) should be considered for patients with refractory ILD. Patients with anti-ARS antibody-positive ILD respond better to GC treatment, but with frequent recurrence; thus, GCs plus immunosuppressants (TAC, CsA, azathioprine, and mycophenolate mofetil) are often needed in order to achieve favorable long-term disease control. PM/DM/CADM-ILD management is still a therapeutic challenge for clinicians, as evidence-based guidelines do not exist to help with management decisions. A few prospective clinical trials have been recently reported regarding the treatment of PM/DM/CADM-ILD. Here, the current knowledge on the pharmacologic managements of PM/DM/CADM-ILD was mainly reviewed.

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Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Respiration ◽

10.1159/000488358 ◽

2018 ◽

Vol 96 (3) ◽

pp. 210-221 ◽

Cited By ~ 8

Author(s):

Hideaki Yamakawa ◽

Eri Hagiwara ◽

Hideya Kitamura ◽

Tae Iwasawa ◽

Ryota Otoshi ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Predictive Factors ◽

Trna Synthetase ◽

Aminoacyl Trna Synthetase

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Morbidity and mortality following surgical lung biopsy in interstitial lung disease in a specialized multidisciplinary center

10.1183/13993003.congress-2021.pa2238 ◽

2021 ◽

Author(s):

Vanessa Menezes ◽

Clare Pollock ◽

Adéline Jouquan ◽

Julie Morisset ◽

Charles Leduc ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Lung Biopsy ◽

Morbidity And Mortality ◽

Surgical Lung Biopsy

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Direct Comparison Between Pathological Diagnosis of Cryobiopsy and Surgical Lung Biopsy for Interstitial Lung Disease

10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a4320 ◽

2019 ◽

Author(s):

T. Baba ◽

T. Takemura ◽

K. Okudela ◽

M. Asaoka ◽

T. Katano ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Lung Biopsy ◽

Pathological Diagnosis ◽

Surgical Lung Biopsy

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Concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in patients with diffuse interstitial lung disease

Diagnostic Pathology ◽

10.1186/s13000-019-0908-z ◽

2019 ◽

Vol 14 (1) ◽

Cited By ~ 3

Author(s):

Yoshiaki Zaizen ◽

Yasuo Kohashi ◽

Kishio Kuroda ◽

Kazuhiro Tabata ◽

Yuka Kitamura ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Lung Biopsy ◽

Usual Interstitial Pneumonia ◽

Airway Disease ◽

Surgical Lung Biopsy ◽

High Concordance ◽

Transbronchial Lung Cryobiopsy

Abstract Background Increasing evidence indicates the utility of transbronchial lung cryobiopsy (TBLC) for the diagnosis of interstitial lung disease (ILD). However, only one study has compared TBLC and surgical lung biopsy (SLB) performed on the same patients. Methods We identified seven patients with ILD with TBLC and SLB. We evaluated the clinical characteristics and made a pathological diagnosis based on the official ATS/ERS/JRS/ALAT clinical practice guideline of idiopathic pulmonary fibrosis with both TBLC and SLB. Results Six cases were diagnosed as Usual interstitial pneumonia (UIP) in both TBLC and SLB. One case was diagnosed as indeterminate for UIP with TBLC and probable UIP with SLB. Etiological diagnosis with TBLC and SLB were concordant in 2 cases of idiopathic pulmonary fibrosis (IPF) but discordant for other diagnoses. Major histological findings of UIP including dense fibrosis, peripheral distribution, and fibroblastic foci showed high concordance between TBLC and SLB, which implies that TBLC can reliably detect these features. In contrast, loose fibrosis, cellular infiltration, and airway disease showed poor concordance between the two methods. Conclusion Our study showed that TBLC is useful for UIP diagnosis but not for other ILD. With a multidisciplinary approach, diagnosis of IPF may be determined by TBLC, whereas ILD other than IPF may require SLB.

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