Tofacitinib in pyoderma gangrenosum - A case series.

Pyoderma gangrenosum (PG) is a rare autoinflammatory disorder falling under the spectrum of neutrophilic dermatosis, characterized by distinctive skin ulceration which is non-infective, non-neoplastic and usually with no primary vasculitis. PG lesions are notorious for relapse and hence require multiple trials of medications often with prolonged and concomitant use of steroids. Due to lack of evidence-based studies on effective treatment options for PG, we have presented three isolated biopsy-proven PG cases who were successfully treated with Tofacitinib, a Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway inhibitor, without relapse in follow up.

Uncommon presentation of granulomatosis with polyangiitis: Prostate involvement.

Granulomatosis with polyangiitis (GPA) is an autoimmune granulomatous disease of unknown etiology; frequently associated with anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA). Although any organ can be involved, prostatic involvement in GPA is very rare. We present a male patient with GPA, 26 years old, with pulmonary manifestations and prostatic involvement who underwent exhaustive evaluation. The patient's laboratory tests and imaging scans showed evidence of lesions in multiple areas, including the prostate. Histopathological testing confirmed that the lesions were consistent with granulomatosis with polyangiitis. The patient was treated with oral steroids and rituximab and showed significant improvement. He was later maintained on azathioprine without any relapse.

Tofacitinib as monotherapy in cutaneous polyarteritis nodosa: a case series.

Objective: Cutaneous polyarteritis nodosa (CPAN) is a distinct clinical entity represented by a chronic, relapsing, benign course, with rare systemic involvement. Treatment is with CSs, CYC or other conventional synthetic DMARDs (csDMARDs). In this case series, we aimed to share our varied clinical experience of successfully treating patients with CPAN, with tofacitinib in a refractory/relapsing course or as upfront monotherapy without CSs/csDMARDs. Methods: We report this retrospective case series managed at our rheumatology centre in Bangalore from 2019 to 2022. Four patients identified as CPAN on biopsy were able to achieve disease-free remission with tofacitinib as part of their treatment, with no relapse on further follow-up. Our patients presented with subcutaneous nodules and cutaneous ulcers. After systemic evaluation, all the patients underwent skin biopsy, which showed fibrinoid necrosis in the vessel walls of the dermis, with a histopathological impression of CPAN. They were initially treated with a conventional approach of CSs with/without csDMARDs. On experiencing a refractory/relapsing course, tofacitinib was tried in all the patients as either CS sparing or upfront monotherapy without concomitant csDMARDs. Results: Use of tofacitinib resulted in improvement of ulcers and paraesthesia and in gradual healing of skin lesions, albeit with scarring, with no further recurrence or relapse over a follow-up period of 6 months for all the patients. The therapeutic effect of tofacitinib was consistent when used either as CS sparing or as upfront monotherapy, thereby proving the drug to be a promising option that warrants larger trials in future to treat the subset of patients with established CPAN. Conclusion: Tofacitinib could be used for disease-free remission as monotherapy for CPAN either upfront or as CS sparing, even without concomitant csDMARDs, in those patients who are dependent on CSs or multiple DMARDs.

Real-life Tolerability and Effectiveness of Adalimumab Biosimilar in Ankylosing Spondylitis: the Adalimumab Biosimilar Patient Registry Data.

OBJECTIVE: Adalimumab is a well-established anti-tumor necrosis factor therapy for patients with ankylosing spondylitis (AS). An indigenously developed biosimilar adalimumab (bADA) (ZRC-3197; Exemptia) is approved for prescribing in India. In this article, we present the effectiveness and tolerability of this bADA in real-life Indian patients with AS from the Adalimumab Biosimilar Patient Registry (ASPIRE) (ISRCTN: 16838474). METHODS: ASPIRE is a postmarketing observational registry for evaluating the real-world experiences of patients with autoimmune inflammatory disorders across multiple centers in India who were prescribed 40 mg of Exemptia subcutaneously every fortnight. For this report, data available until 24 weeks of bADA treatment for patients with AS who were included in the registry were evaluated. RESULTS: Data from 308 patients with AS from the registry (median age of 35.0 [range 17-68] years, 19% women) were analyzed. In analyzable patients with complete data, there was a gradual and significant decrease (P < 0.001) in the primary disease outcome scores (the mean Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] score [n = 107] improved from 6.2 ± 1.54 to 2.1 ± 0.64, and the median visual analogue scale [VAS] score [n = 101] improved from 8 to 2) after 24 weeks of bADA therapy. BASDAI score was lower than 4 in about 94% of patients after 24 weeks of therapy, and 95% of patients achieved BASDAI50 response. The overall global assessment for efficacy and tolerability was 'good' to 'excellent' for a majority of the patients (≥98%), as rated by physicians as well as patients. The therapy was tolerated well, and there were no new unexpected adverse reactions with the biosimilar's use during this study. CONCLUSION: This report demonstrates the tolerability and effectiveness of bADA (Exemptia) after its clinical use for 24 weeks in real-world patients with AS from Indian clinical practice.