Improvement of salt and pepper skin changes in diffuse scleroderma after treatment with tofacitinib
Year: 2022
Type: article
Study design: Case Report
Abstract: We present the case of a 48-year-old woman with a history of diffuse cutaneous systemic sclerosis (SSc) with prominent salt and pepper changes.In August 2015, she was diagnosed with SSc based on proximal skin thickening, Raynaud's phenomenon with digital ulcers, abnormal nailfold capillary changes (giant capillaries and hemorrhages), esophageal dysmotility, and calcinosis.At the time of diagnosis, she also developed proximal muscle weakness and creatine kinase (CK) was elevated to 600 IU/L.She was prescribed prednisone and mycophenolate mofetil (MMF), which she intermittently took from 2015-2017.She was referred to our Center by November 2018 for progressive muscle weakness and skin tightening and was not on any therapy.On skin examination, her modified Rodnan skin score (mRSS) was 17 and she had prominent salt and pepper like changes on the anterior chest, forehead, and posterior neck.Muscle strength exam revealed proximal muscle weakness with neck flexors of 2/5, bilateral arm abductors 3/5, and hip flexors 2/5 on MRC scale.The remainder of the examination was unremarkable.CK was 142 IU/L.SSc specific antibodies were negative.Myositis panel was positive for U3-RNP (anti-fibrillarin).She underwent electromyography (EMG) which demonstrated severe non-irritable myopathy and bilateral thigh muscle magnetic resonance imaging (MRI-T2/STIR) demonstrated muscle edema.She declined muscle biopsy.Based on active skin disease and severe myopathy, MMF was initiated in December 2018 but ultimately discontinued due to gastrointestinal side effects.Intravenous immunoglobulin (IVIG) was started in March 2020 and within 4 months, she had improvement of her proximal muscle weakness.By November 2020, she developed inflammatory arthritis and was started on tofacitinib 11mg XR daily.Three months thereafter, in January 2021, she reported improvement in inflammatory arthritis, skin tightening, and hypopigmentation.On follow-up examination in August 2021, she had significant improvement of the salt and pepper changes of the skin (Figure 1).Interestingly, there was also improvement of the mRSS (17 points to 8 points) and muscle strength (2/5 to 4+/5 in proximal muscles).Janus kinase (JAK) inhibitors such as tofacitinib have been reported to be efficacious in treating diffuse SSc and dermatomyositis in early phase clinical trials (1, 2).Bariticinitb has also been reported to be efficacious in the treatment
Institutions: Johns Hopkins University, Johns Hopkins Medicine
Language: English
Subfield: Pathology and Forensic Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4300690896