Correlation Between the Change of Peripheral Lymphocyte Subsets and Clinically Amyopathic Dermatomyositis Combined With Rapidly Progressive Interstitial Lung Disease

Acute respiratory distress syndrome (ARDS) occurs in Clinically Amyopathic Dermatomyisitis(CADM) combined with Rapidly Progressive Interstitial Lung Disease(RPILD) within 1-3 months, which leads to death of patients and is difficult to treat. Even if high doses of glucocorticoids are ineffective, there is no recommended treatment for such patients, which is a huge medical challenge.Lymphopenia is an independent risk factor for death in CADM-RPILD, but the cause of lymphopenia is unclear.In this study, the level of lymphocyte subsets in peripheral blood was detected by flow cytometer, in order to further clarify the pathogenesis of the disease, to facilitate clinical guidance of treatment, and to improve the survival rate of patients.In addition, studies have shown that INF-α levels are significantly increased in CADM patients combined with RPILD and are a poor prognostic factor for CADM-RPILD, suggesting that the interferon system plays a role in the pathogenesis of CADM and can be used as an evaluation index of the severity of CADM-RPILD.In this study, the levels of relevant cytokines including INF and IL-2, IL-17, IL-18, IL-6 were detected simultaneously, and the relationship between disease activity and lymphocyte subsets was analyzed, and the changes of lymphocyte subsets after Tofacitinib treatment were determined in order to facilitate clinical guidance of treatment.

Study Overview

• Status: Recruiting
• Conditions: Clinically Amyopathic Dermatomyisitis(CAMD)
• Intervention / Treatment: Drug: Tofacitinib 5 MG [Xeljanz]

• Study Type: Observational
• Enrollment (Anticipated): 80

Contacts and Locations

• Study Contact: Name: Yanfeng Hou; Phone Number: +8615168888165; Email: yfhou1016@163.com

Study Locations

• China
  • Shandong
    • Jinan, Shandong, China, 252000
      • Recruiting
      • Qianfoshan Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 80 years (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patient with Clinically Amyopathic Dermatomyisitis combined with Rapidly Progressive Interstitial Lung Disease

Description

Inclusion Criteria:

1. Age≥18 years old,≤80years old,no gender limit;
2. CADM of diagnosis according to Sontheimer diagnostic criteria;
3. combined with Rapidly Progressive Interstitial Lung Disease

Exclusion Criteria:

1. Age<18years old，>80years old;
2. potential malignant tumor

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
The level of a lymphocyte in a lymphocyte subset correlates with disease activity.	3 month

Collaborators and Investigators

• Sponsor: Jing Liang

Study record dates

Study Major Dates

• Study Start (ACTUAL): October 1, 2020
• Primary Completion (ANTICIPATED): April 30, 2023
• Study Completion (ANTICIPATED): April 30, 2023

Study Registration Dates

• First Submitted: September 15, 2020
• First Submitted That Met QC Criteria: October 27, 2020
• First Posted (ACTUAL): November 3, 2020

Study Record Updates

• Last Update Posted (ACTUAL): November 3, 2020
• Last Update Submitted That Met QC Criteria: October 27, 2020
• Last Verified: September 1, 2020

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Nervous System Diseases; Skin Diseases; Respiratory Tract Diseases; Musculoskeletal Diseases; Connective Tissue Diseases; Muscular Diseases; Neuromuscular Diseases; Polymyositis; Myositis; Lung Diseases; Lung Diseases, Interstitial; Dermatomyositis; Molecular Mechanisms of Pharmacological Action; Enzyme Inhibitors; Protein Kinase Inhibitors; Tofacitinib
• Other Study ID Numbers: TY017AN

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: Yes