The Therapeutic Efficacy of Abatacept for Rheumatoid Arthritis-Associated Interstitial Lung Disease: Insights from a 12-Month Trial Using Semi-Quantitative Chest High-Resolution Computed Tomography Imaging.

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a major complication of rheumatoid arthritis (RA), but effective treatment remains an unmet need in its management. Our aim was to evaluate the therapeutic efficacy of abatacept for RA-ILD. This observational retrospective study included patients with RA-ILD treated with abatacept between 2012 and 2021.

Aggressive multi-combination therapy for anti-MDA5 antibody-positive dermatomyositis-rapidly progressive interstitial lung disease.

Objectives: To elucidate the efficacy and safety of aggressive multi-combination therapy with mycophenolate mofetil, rituximab, and plasma exchange or polymyxin B immobilized fiber column direct hemoperfusion followed by conventional therapy with corticosteroids, calcineurin inhibitors, and intravenous pulse cyclophosphamide in patients with rapidly progressive interstitial lung disease (RPILD) with anti-melanoma differentiation-associated gene 5 (MDA5)-antibody-positive dermatomyositis (DM).

Methods: A total of 23 patients with anti-MDA5 antibody-positive DM-RPILD were enrolled, with nine patients in Group A (treated conventionally before March 2015) and 14 patients in Group B (received aggressive treatment after April 2015).

Results: Pretreatment severity of interstitial lung disease (ILD) did not differ between the two groups.

Examination of nailfold videocapillaroscopy findings in ANCA-associated vasculitis.

Objective: The objective of this study was to evaluate nailfold videocapillaroscopy (NVC) as a useful tool for assessing the disease activity of ANCA-associated vasculitis (AAV).

Methods: This study enrolled 51 patients with AAV and 21 healthy controls. We scored NVC findings semiquantitatively, and compared them between AAV patients and controls.

Comparison of therapeutic effects of combination therapy with prednisolone and tacrolimus or azathioprine on progressive interstitial pneumonia with systemic sclerosis.

Objectives: We retrospectively compared the therapeutic effects of combination therapy with prednisolone (PSL) and oral tacrolimus (TAC) or azathioprine (AZA) on progressive interstitial pneumonia with systemic sclerosis (SSc-PIP).

Methods: The effects of PSL (0.2-0.

CCL2 produced by CD68+/CD163+ macrophages as a promising clinical biomarker of microscopic polyangiitis-interstitial lung disease.

Objectives: Microscopic polyangiitis (MPA) is often complicated by interstitial lung disease (ILD); however, biomarkers that can be used to diagnose and predict the progression of MPA-ILD have not been identified. In this study, we evaluated various serum biomarkers in MPA-ILD to assess their diagnostic and predictive performance.

Methods: We enrolled 49 patients with anti-neutrophil cytoplasmic antibody (ANCA)+ MPA and 10 healthy controls, with 32 of the MPA patients also presenting ILD.

Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease.

The prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory, and radiological findings, treatments, and outcomes of 80 patients with MPA, and investigated prognostic factors of respiratory-related death in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive MPA-ILD.

The comparison of nailfold videocapillaroscopy findings between anti-melanoma differentiation-associated gene 5 antibody and anti-aminoacyl tRNA synthetase antibody in patients with dermatomyositis complicated by interstitial lung disease.

Dermatomyositis (DM) is frequently complicated by interstitial lung disease (ILD), which increases mortality. This study aims to elucidate the clinical significance of nailfold videocapillaroscopy (NVC) on assessing the disease activity and prognosis of DM-ILD. We compared the NVC findings between anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and anti-aminoacyl tRNA synthetase (anti-ARS) antibody-positive patients, the survival and ILD-related death groups, and examined the association of NVC findings with prognostic factors of DM-ILD.

Exploration of pathomechanism using comprehensive analysis of serum cytokines in polymyositis/dermatomyositis-interstitial lung disease.

Objectives: To elucidate the serum cytokine profile and address the pathomechanism of interstitial lung disease (ILD) complicated with PM/DM.

Methods: Forty patients with PM/DM-ILD were enrolled, and principal components analysis and cluster analysis were performed to classify patients into subgroups. Additionally, we compared cytokine profiles between the survivors and dead patients and between anti-melanoma differentiation-associated gene 5 antibody- and anti-aminoacyl tRNA synthetase antibody-positive ILD patients.

Efficacy and safety of oral high-trough level tacrolimus in acute/subacute interstitial pneumonia with dermatomyositis.

Objectives: We assessed the efficacy and safety of combination therapy with glucocorticoids and high-trough level tacrolimus (TAC) for the treatment of acute/subacute interstitial pneumonia (A/SIP) in patients with dermatomyositis (DM).

Methods: Eleven DM-A/SIP patients were enrolled. The combination therapy with glucocorticoids and TAC was started as early as possible after DM-A/SIP was diagnosed.

Potential of Krebs von den Lungen-6 as a predictor of relapse in interstitial pneumonia with anti-aminoacyl tRNA synthetase antibodies-positive dermatomyositis.

Objective: To identify a predictor of relapse in interstitial pneumonia (IP) in patients with anti-aminoacyl tRNA synthetase antibodies-positive dermatomyositis (ARS-DMIP).

Methods: This retrospective cohort study comprised 27 ARS-DMIP patients. We compared clinical and laboratory findings between the relapse and non-relapse groups during 2 years after treatment initiation to find predictors of relapse in IP.

Efficacy and safety of combination therapy with prednisolone and oral tacrolimus for progressive interstitial pneumonia with systemic sclerosis: A retrospective study.

Objectives: We retrospectively investigated efficacy and safety of combination therapy with prednisolone (PSL) and tacrolimus (TAC) for progressive interstitial pneumonitis with systemic sclerosis (SSc-PIP).

Methods: We studied 11 patients with SSc-PIP who received combination therapy with PSL (0.5 mg/kg/d) and TAC (3 mg/d).

[Granulomatosis with Polyangiitis Complicated with Gastrointestinal Perforation: A Case Report and Review of Literature].

  A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes.

Evaluation of clinical prognostic factors for interstitial pneumonia in anti-MDA5 antibody-positive dermatomyositis patients.

Objectives: We retrospectively investigated clinical prognostic factors for interstitial pneumonia (IP) in anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive dermatomyositis (DM) patients.

Methods: Subjects comprised 18 patients with anti-MDA5 Ab-positive DM-IP (9 survivors; 9 deaths).

Results: Initial serum albumin levels, ferritin levels, and ground-glass opacity (GGO) scores in the right middle lobes were significantly higher in the death group than in the survivor group (p = .

Chemokine profiles of interstitial pneumonia in patients with dermatomyositis: a case control study.

Chemokines play an important role in the pathophysiology of dermatomyositis (DM) with interstitial pneumonia (IP). However, the relation between chemokines and the disease activity or prognosis of DM-IP has not been elucidated. We evaluated the serum C-C motif chemokine ligand (CCL) 2, Th1 chemokines (C-X-C motif chemokine ligand [CXCL] 9, CXCL10, CXCL11), and Th2 chemokine (CCL17) profiles of 30 patients, and examined the relation between these chemokines and the disease activity or prognosis of DM-IP.

Comparison of long-term prognosis and relapse of dermatomyositis complicated with interstitial pneumonia according to autoantibodies: anti-aminoacyl tRNA synthetase antibodies versus anti-melanoma differentiation-associated gene 5 antibody.

The aim of this study was to investigate long-term prognosis and relapse of dermatomyositis complicated with interstitial pneumonia (DMIP) according to anti-aminoacyl tRNA synthetase (ARS) antibodies and anti-melanoma differentiation-associated gene 5 (MDA5) antibody. This retrospective study comprised 36 patients with DMIP who were divided into the anti-ARS antibody-positive group (ARS+) (n = 12), anti MDA5 antibody-positive group (MDA5+) (n = 11), double-negative group (ARS-/MDA5-) (n = 11), and double-positive group (ARS+/MDA5+) (n = 1). Clinical features, treatment, prognoses, and relapses during the 2 years after initiation of treatment were compared between three groups excluding ARS+/MDA5+ group.

Chylothorax in dermatomyositis complicated with interstitial pneumonia.

Chylothorax is a disease in which chyle leaks and accumulates in the thoracic cavity. Interstitial pneumonia and pneumomediastinum are common thoracic manifestations of dermatomyositis, but chylothorax complicated with dermatomyositis is not reported. We report a case of dermatomyositis with interstitial pneumonia complicated by chylothorax.

The proton pump inhibitor lansoprazole, but not rabeprazole, the increased blood concentrations of calcineurin inhibitors in Japanese patients with connective tissue diseases.

Objective: Proton pump inhibitors (PPIs) are frequently coadministered with calcineurin inhibitors (CNIs) such as tacrolimus (TAC) and cyclosporin A (CSA), to treat or prevent upper gastrointestinal complications in Japanese patients with connective tissue diseases (CTDs). The coadministration of PPIs increases the blood concentration of TAC due to drug interaction. We retrospectively investigated the influence of the coadministration of PPIs and CNIs, as well as the influence of the cytochrome P450 (CYP) 2C19 gene polymorphism status, on the blood concentrations of TAC and CSA in patients with CTDs.

Pre-treatment ferritin level and alveolar-arterial oxygen gradient can predict mortality rate due to acute/subacute interstitial pneumonia in dermatomyositis treated by cyclosporine a/glucocorticosteroid combination therapy: a case control study [corrected].

Background: Acute/subacute interstitial pneumonia in dermatomyositis (DM-A/SIP) is a disease associated with a poor prognosis that resists treatment with glucocorticosteroids (GC) and progresses rapidly in a period of weeks to months to death. We retrospectively studied outcomes, prognostic factors, and their relations with survival rate in patients with DM-A/SIP treated with early cyclosporine A (CSA)/GC combination therapy and 2-hour postdose blood concentration monitoring.

Methods: This study comprised 32 DM-A/SIP patients who were simultaneously treated with CSA and prednisolone.

Ultrasound evaluation of the effects of leukocytapheresis on rheumatoid arthritis.

Leukocytapheresis (LCAP) is effective in treating rheumatoid arthritis (RA). Ultrasound (US) examination of joints is useful for evaluating disease activity and therapeutic effects in RA, but the clinical assessment of LCAP therapy with US has been little reported. We investigated the usefulness of US for evaluating the effects of LCAP in patients with RA.

Combination with corticosteroids and cyclosporin-A improves pulmonary function test results and chest HRCT findings in dermatomyositis patients with acute/subacute interstitial pneumonia.

We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin-A (CSA) on the findings of pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCTs) scans in patients with dermatomyositis (DM) and acute/subacute interstitial pneumonia (A/SIP). We also examined whether CSA therapy improved PFT and chest HRCT findings. DM patients (n=14) with A/SIP were treated with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA within 4.

[Effective treatment of interstitial pneumonia with tacrolimus in a patient with dermatomyositis who was intolerant to cyclosporin-A].

A 67-year-old woman was admitted to our hospital because of erythema in the eyelids and fingers of both hands. Slight muscle weakness in the proximal limbs, a heliotrope rash, Gottron's sign, and mechanic's hand were observed. Creatine kinase serum levels were slightly elevated (376U/l), and the patient tested negative for all autoantibodies except for the antinuclear antibody.

Microscopic polyangiitis complicated with cerebral infarction and hemorrhage: a case report and review of literature.

We report a case of MPA with cerebral infarction and hemorrhage. A 72-year-old man was admitted to our hospital because of high fever, speech failure, and weakness of the left limbs in April 2008. Magnetic resonance imaging of the head showed cerebral infarction at the right corona radiata.