¹Akil Parmar ¹Beena Hameed

¹Kings College Hospital

Purpose Statement: Introduction

Systemic sclerosis is a rare autoimmune disease characterized by several key features. These include small vessel vasculopathy, immune dysregulation, and fibrosis of both the skin and internal organs. Importantly, systemic sclerosis has implications for life expectancy, with a standardized mortality ratio estimated to be between 1.5 and 7.2 [1]. Limited cutaneous scleroderma gradually affects the skin on the fingers, hands, face, lower arms, and below the knees. In contrast, diffuse cutaneous scleroderma develops rapidly, initially impacting fingers and toes but spreading to the upper arms, trunk, or thighs, often leading to more significant internal organ damage. Diagnosis relies on clinical features, emphasizing early detection, extent of skin involvement and continuous monitoring for internal organ complications. While no definitive treatment exists, management with anti-rheumatic drugs has shown efficacy in some aspects of disease. Additionally, systemic sclerosis is associated with an increased risk of cancer, suggesting a potential paraneoplastic syndrome.

Method(s): We present a 57-year-old non-smoker female of American descent, who developed pain and stiffness of hands, arms, feet and legs in August 2023. It gradually became worse. She noticed progressive stiffness of hands, arms, and legs. She also developed gastroesophageal reflex and reduced appetite but no nausea, vomiting or abdominal pain. No history of weight loss. Additionally, she noticed her fingers turn pale in response to cold for few minutes followed by bluish discoloration for another few minutes.  She had no history of digital ulcerations, shortness of breath or cough.

Past medical history was significant of lung adenocarcinoma, for which she had excision of 30% of lung on left side in September 2022.

She also had sleep apnea, anxiety, and depression.

Her regular medications included alprazolam 0.5 mg 3 times a day, duloxetine 60 mg a day.

On examination she was found to have loss of skin creases of her fingers. She was unable to make a tight fist.  She had sclerodactyly and sclerosis of skin involving both upper and lower limbs extending above elbow and knees Joints with modified rodnan score of 24. Her chest was clear to auscultation. There was no extra-cardiac sound on cardiac auscultation.

She had a skin biopsy showing early signs of morphea.

Further investigations revealed, a negative anti-nuclear antibody with a positive anti-centromere antibody and anti Mi – 2 Alpha antibody. Rheumatoid factor and anti-citrullinated antibodies. C reactive protein was 42, C3 and C4 normal.

Lung function test and ECHO cardiogram was normal. A repeat CT scan chest did not show any recurrence of lung cancer. She was started on oral prednisolone 15mg per day with mycophenolate Mofetil in December 2023. She also was given proton pump inhibitor esomeprazole 40mg a day with nifedipine 10mg twice a day.

Result(s): Since then, her joint pain and stiffness improved remarkably and so did her Raynaud’s and gastric reflux symptoms.  Skin modified rodnan score remained stable at 24.

Conclusion(s): The literature extensively addresses the association between systemic sclerosis and cancer, emphasizing the susceptibility of individuals with scleroderma to various cancer types. Notably, research predominantly focuses on the increased cancer risk in scleroderma patients, particularly in males, and the elevated likelihood of lung, bladder, hematologic, and liver cancers. Limited literature explores the reverse relationship, investigating how cancer may contribute to the development of systemic sclerosis. A meta-analysis shows a significant 75% [3] heightened likelihood of cancer in individuals with scleroderma compared to the general population. Interestingly, our case presents a unique scenario where a patient initially treated for lung cancer later developed diffuse systemic sclerosis post excision of lung adeno-carcinoma with no recurrence.