Characterization of SSc-ILD patients and linkage to clinical outcomes

The purpose of this study is to better understand the development of scleroderma-related lung fibrosis (scarring)/ interstitial lung disease (ILD) and how certain molecular and cellular characteristics of scleroderma lung disease may be linked to clinical outcomes. Depending on your diagnosis date, study procedures may include screening form and questionnaires, physical examination, blood collection, high resolution computed tomography (hrCT), skin biopsy collection, and suture removal.

Eligibility criteria:

In order to be eligible for inclusion in the study, an individual must meet all of the following criteria:

• Yale scleroderma/systemic sclerosis patient
• Must be ≥ 18 years old and meet the 2013 American College of Rheumatology criteria for the diagnosis of systemic sclerosis (diffuse or limited)
• Receiving clinical care at Yale in the Rheumatology and/or Pulmonary clinics

Any individual who meets any of the following criteria will be excluded from participation in this study:

• Diagnosed with an overlap syndrome (i.e., SLE, RA, etc.) Overlap with polymyositis and/or Sjogren’s syndrome are permitted
• Unable to provide informed consent.
• Currently pregnant.
• Current smoker or former smoker (> 10 pack years; pack years= # packs of cigarettes smoked per day (PPD) * # years smoked; i.e., 1 PPD * 3 years= 3 pack years, ½ PPD * 2 years= 1 pack year)
• Leukopenia (WBC < 4000 cells/μL)
• Anemia (Hg < 8g/dl)
• Comorbidities of uncontrolled congestive heart failure, cancer, HIV, chronic liver disease
• Known blood disorders or treatments that may result in excessive bleeding from skin biopsies (hemodialysis, severe thrombocytopenia (platelet count <50,000/µL), supratherapeutic INR from warfarin)
• Presence of skin diseases that would alter gene expression (psoriasis or morphea) or result in excessive scarring following skin biopsies (h/o keloid formation)
• History of lung diseases (asthma, emphysema, chronic bronchitis, recurrent pneumoniaAllergies to lidocaine or epinephrine