CRA16 A DOUBLE TROUBLE: BILATERAL SPONTANEOUS PNEUMOTHORAX AS THE FIRST SIGN OF SYSTEMIC SCLEROSIS RELATED ILD

Frances Kaviarasan A/L Charles, Sunita Devi Hukam Gopal Chand, Anandprakash Sivaprakasam, Ng Boon Han
Hospital Sultan Abdul Halim, Kedah, Malaysia

Introduction
Spontaneous bilateral pneumothorax is a rare but serious complication of systemic sclerosis-related lung disease. It can sometimes be the first sign of an underlying autoimmune condition, making early diagnosis important to guide treatment.

Objectives
To share a case of a woman who presented with sudden breathlessness due to bilateral pneumothorax and was later diagnosed with diffuse cutaneous systemic sclerosis complicated by interstitial lung disease.

Case Report
A 54-year-old Malay woman with hypertension and no history of smoking presented with sudden breathlessness and low oxygen saturation. Imaging showed a right pneumothorax which required chest tube insertion, followed by detection of a left pneumothorax the next day requiring a second chest tube. She had a history of chronic dry cough, skin tightening, Raynaud’s phenomenon, and significant weight loss. Serology showed positive ANA and strong anti-Scl-70 antibodies. CT thorax demonstrated lung fibrosis, bullae, and bronchiectasis. Surgery for bullae removal done and lung biopsy confirmed interstitial fibrosis with features suggestive of both usual interstitial pneumonia (UIP) and non specific interstitial pneumonia (NSIP).

Treatment and Outcome
She was treated with mycophenolate mofetil and hydroxychloroquine and showed good recovery post-surgery. She remains under close follow-up with respiratory and rheumatology teams.

Conclusion
This case illustrates that spontaneous bilateral pneumothorax can be an unusual first presentation of systemic sclerosis-related lung disease. Early recognition and multidisciplinary management are key to better patient outcomes.