No cure, but patient-specific Tx can improve quality of life
SSc greatly reduces a patient’s self-esteem and quality of life. Because there is no cure, many patients develop depression.11,12 However, depending on the patient, some treatments can control symptoms and complications, and thus improve quality of life.
Because SSc is an autoimmune disease, immunosuppressive agents are a pillar of treatment. Recent studies have shown that low-dose pulse cyclophosphamide can stabilize pulmonary function.13
Raynaud’s phenomenon is treated with low-dose calcium-channel blockers, such as amlodipine 5 mg/d that is gradually increased to as much as 20 mg/d to increase blood flow to the fingers.14
Fibrosis of the skin is treated with daily doses of PUVA (photochemotherapy) 0.25 J/cm2 or 0.4 J/cm2 for 3 to 8 weeks (total doses between 3.5 J/cm2 and 9.6 J/cm2); this results in improvements in hand closure, skin sclerosis index, and flexion of fingers or knee joints.15 D-penicillamine, an anti-fibrotic drug that has the ability to not only destabilize tissue collagen but also reduce its production, is started early with a low dose and then carefully increased.16
Our patient declined hospital care or treatment and refused referral to a physiotherapist for hand exercises, paraffin baths, massages, splints, or water aerobics. Her condition remained stable and she has been able to manage on her own, despite her advanced stage of CREST syndrome.
CORRESPONDENCE
Chandramani Thuraisingham, MBBS, FAFP, FRACGP, AM, DRM, PDOH, International Medical University, Clinical School Seremban, Jalan Rasah, 70300 Seremban, Negeri Sembilan, Malaysia; chandramani@imu.edu.my.