[A case of systemic sclerosis]

Area of Science:

• Immunology
• Rheumatology
• Oncology

Background:

• Anti-RNA polymerase III antibodies (ARNpol3) are linked to severe systemic sclerosis (SSc), characterized by diffuse cutaneous involvement, renal crisis, and poor survival.
• Pulmonary involvement is less common in ARNpol3-positive SSc, making its presentation a significant clinical observation.

Observation:

• A case study of a woman initially diagnosed with drug-induced interstitial lung disease.
• The patient later presented with chronic interstitial pneumonia exhibiting autoimmune features.
• The disease progressed through three stages, culminating in rapidly progressive diffuse skin sclerosis and ARNpol3 positivity.

Findings:

• The patient's condition evolved to include diffuse cutaneous systemic sclerosis with ARNpol3 antibodies.
• Ovarian cancer remission was noted in the context of the patient's evolving autoimmune disease.
• This case illustrates a rare presentation of ARNpol3-positive SSc with concurrent malignancy and interstitial lung disease.

Implications:

• Highlights the complex interplay between autoimmune diseases, malignancy, and specific autoantibodies like ARNpol3.
• Suggests a need for vigilant cancer screening in patients presenting with ARNpol3-positive SSc and interstitial pneumonia.
• Underscores the importance of recognizing atypical presentations of systemic sclerosis for timely diagnosis and management.