Treatment Approach for Systemic Sclerosis and Cold Urticaria

Initial Evaluation and Screening

Patients who are positive for anti-topoisomerase I (Scl-70) antibodies have a higher frequency of interstitial lung disease (ILD) and require thorough screening with history, physical examination, chest radiography, pulmonary function testing, and high-resolution CT of the lungs 1

Cold exposure is a well-documented trigger for Raynaud's phenomenon in systemic sclerosis patients, with patients reporting more frequent and longer exacerbations during winter 2
For cold-triggered hives (cold urticaria) and Raynaud's phenomenon, non-pharmacological interventions such as use of gloves and heating devices for the hands, avoidance of direct contact with cold surfaces, thorough drying of the skin after exposure to moisture, and avoidance of sudden temperature changes can be implemented 2

The American College of Rheumatology recommends mycophenolate mofetil (MMF) as first-line therapy for SSc-ILD, with methotrexate considered if musculoskeletal involvement is predominant [4] [5]
Tocilizumab, rituximab, and nintedanib can be considered as second-line options for SSc-ILD treatment, particularly in early inflammatory disease or progressive fibrosing ILD 5
Second-generation H1-antihistamines are the first-line treatment for cold urticaria, with dosage increased up to four times the standard dose for refractory cases, and omalizumab considered for antihistamine-resistant cases 5

Regular monitoring of pulmonary function tests, especially in early diffuse cutaneous SSc, is recommended by the European Respiratory Society 1
Blood pressure monitoring to detect early scleroderma renal crisis, and screening for other organ involvement including cardiac, gastrointestinal, and musculoskeletal manifestations are also recommended 1

Avoid glucocorticoids in patients with early diffuse cutaneous SSc due to increased risk of scleroderma renal crisis, as advised by the American College of Rheumatology 1
Physical exercise should be encouraged as it improves outcomes in SSc patients, despite cold-induced symptoms, according to the European League Against Rheumatism 2

Consider referral to pulmonology if ILD is detected, as recommended by the American Thoracic Society 1
Dermatology consultation may be helpful for management of refractory cold urticaria, and physical and occupational therapy can improve functional impairment in SSc patients, as suggested by the British Society for Rheumatology [2] [3]