Successful use of tofacitinib in the treatment of diffuse systemic sclerosis and axial spondyloarthritis: a case-based review

Kyriakou, Avgoustina; Parperis, Konstantinos; Nikiphorou, Elena; Psarelis, Savvas

doi:10.1007/s00296-020-04767-2

Cited by 9 publications

(11 citation statements)

References 13 publications

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“…Only one patient progressed in their disease on tofacitinib, and the patients treated with tofacitinib improved faster compared to patients treated with cyclophosphamide or mycophenolate mofetil in combination with steroids. An additional case of an HLA‐B27 positive patient with dcSSc and ankylosing spondylitis treated with tofacitinib demonstrated clinical improvement in skin thickness as measured by the mRSS as well as symptom improvement of ankylosing spondylitis 29 . Analogously, a patient with refractory morphea of the legs that failed prednisone 40 mg daily and MTX achieved notable improvement on tofacitinib 10 mg daily for 3 months, both clinically and on skin biopsy 30 …”

Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 98%

“…An additional case of an HLA-B27 positive patient with dcSSc and ankylosing spondylitis treated with tofacitinib demonstrated clinical improvement in skin thickness as measured by the mRSS as well as symptom improvement of ankylosing spondylitis. 29 Analogously, a patient with refractory morphea of the legs that failed prednisone 40 mg daily and MTX achieved notable improvement on tofacitinib 10 mg daily for 3 months, both clinically and on skin biopsy. 30 There are very few reports of the use of JAK inhibitors in children with morphea or juvenile SSc.…”

Section: None Reportedmentioning

confidence: 99%

“…JAK inhibitors represent a new class of disease modifying drugs that have demonstrated notable efficacy in the treatment of a range of dermatologic and rheumatologic diseases such as rheumatoid arthritis (RA), psoriasis, alopecia areata, and dcSSc 29,33–36 . While JAK inhibitors have shown promise for the treatment of multiple dermatologic diseases, there are concerning potential adverse reactions to JAK inhibitors, including hematologic abnormalities, liver and kidney abnormalities with elevated serum creatinine, alanine transaminase (ALT), aspartate transaminase (AST), elevated serum lipids, thromboembolism, increased risk for viral and bacterial infection, and malignancy 37 .…”

Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 99%

“…Overall, studies to date indicate that JAK inhibitors are well tolerated in these patients, with no major adverse events (Table 2). 18,21,23,26–32,38 …”

Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 99%

“…Overall, studies to date indicate that JAK inhibitors are well tolerated in these patients, with no major adverse events (Table 2). 18,21,23,[26][27][28][29][30][31][32]38 5 | CONCLUSION JAK inhibitors have been shown to be efficacious in treating cutaneous thickening in both mouse models of SSc and patients with refractory morphea and SSc, and they have been overall well tolerated.…”

Section: None Reportedmentioning

confidence: 99%

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Janus kinase inhibitors for treatment of morphea and systemic sclerosis: A literature review

Kallis

Benedetto

Thomas

2022

Dermatologic Therapy

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Morphea and systemic sclerosis (SSc) are rare disorders of connective tissue characterized by increased skin thickness and fibrosis, with current treatment options having variable efficacies, many with limited therapeutic benefit. Janus kinase (JAK) inhibitors have been shown in preclinical studies to inhibit the fibrotic pathway in murine models of systemic sclerosis, by blocking TGF-beta mediated pathway of STAT protein activation. Additionally, case reports of the treatment of morphea and SSc with tofacitinib, a JAK 1/3 inhibitor, have shown improvement in skin sclerosis. Several JAK inhibitors have been developed and utilized in dermatologic and rheumatologic diseases. To date, tofacitinib has been by far the most commonly trialed JAK inhibitor in patients with SSc and morphea. Herein we review the preclinical studies reported in the literature supporting the use and efficacy of JAK inhibitors for the treatment of morphea and the cutaneous manifestations of SSc, as well as discuss the clinical cases published to date illustrating the benefits of JAK inhibitors in disease management. The pathogenesis and mechanism of action will be reviewed as it relates to the process of skin fibrosis in morphea and SSc, along with the murine models illustrating efficacy of JAK inhibitors in fibrotic disease. Based on available preclinical and clinical data as well as consideration of the mechanism of action of JAK inhibitors on the pathway for cutaneous fibrosis, there is promising evidence to support the use and further study of JAK inhibitors in the management of morphea and cutaneous fibrosis in SSc.

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Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 98%

Section: None Reportedmentioning

confidence: 99%

Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 99%

“…Overall, studies to date indicate that JAK inhibitors are well tolerated in these patients, with no major adverse events (Table 2). 18,21,23,26–32,38 …”

Section: Preclinical Studies Of Jak Inhibitors In Morphea and Sscmentioning

confidence: 99%