Successful Use of Tofacitinib in Refractory Takayasu Arteritis: A Case Series.

Objective: To study the clinical effectiveness and safety of Tofacitinib in refractory Takayasu arteritis (TAK). Methods: This study was conducted from September 2021 to June 2022. Ten cases of refractory TAK patients were enrolled. TAK patients who required >7.5mg prednisolone or equivalent per day and those who failed to achieve remission despite being on conventional immunomodulators, with an Indian Takayasu Activity Score 2010 (ITAS 2010) of > 1 were included in this study. Tofacitinib was used at a dose of 5 mg twice daily after ruling out latent tuberculosis. The patients were followed up at 1, 3 and 6 months. ESR, CRP and ITAS 2010 were recorded at each visit. Complete blood counts, liver, and kidney function tests were done to assess the adverse effects at baseline and follow up. Results: There was a mean decline in ESR from 60.7 ± 20.05 mm/1st hour at baseline to 11.9 ± 2.38mm/1st hour at 6 months, CRP from 28.9 ± 16.77 mg/L at baseline to 6.8 ± 7.52 mg/L at 6 months, ITAS 2010 from 6.2 ± 2.74 at baseline to 0.6 ± 1.26 at 6 months (p value 0.016). All the patients tolerated tofacitinib well without any adverse effects. Conclusions: The results of our research indicate that tofacitinib is safe and effective for treating patients with refractory TAK.

Successful Use of Tofacitinib in Scleroderma Arthropathy.

Musculoskeletal manifestations of systemic sclerosis (SSc) are frequent and may be one of the early manifestations of the disease. However, arthralgia, pain and stiffness without frank arthritis usually constitute the clinical picture, while overlap syndromes such as rheumatoid-like polyarthritis can dominate when the arthritis is erosive. Hereby, we report a case of primary SSc presenting as frank erosive arthritis involving small and large joints mimicking rheumatoid arthritis, unresponsive to methotrexate, which was successfully treated with tofacitinib.

New-onset systemic vasculitis following SARS-CoV-2 infection and vaccination: the trigger, phenotype, and outcome.

The global health crisis caused by the COVID-19 pandemic overwhelmed the capacity of healthcare systems to cope with the rapidly spreading infection and its associated complications. Among these complications, autoimmune phenomena such as systemic vasculitis emerged as a significant challenge. Both the SARS-CoV-2 virus and the vaccines developed to combat it appeared to induce clinical manifestations resembling various types of systemic vasculitis, affecting large, medium, and small vessels. These virus- or vaccine-induced vasculitides exhibited a distinct natural history and course from de novo vasculitis, as they were more responsive to steroid therapy and some mild cases even resolved spontaneously. Notably, there have been no confirmed cases of SARS-CoV-2 infection or vaccination triggering variable vessel vasculitis like Behcet's disease or Kawasaki disease. IgA vasculitis, which is predominantly a pediatric condition, was more prevalent in adults after COVID-19 infection and they had a favorable outcome with glucocorticoid treatment. The impact of immunosuppression, especially B-cell-depleting agents, on the immunogenicity of the vaccine was evident, but there was no significant increase in the incidence of SARS-CoV-2 infection in these patients compared to the general population. Considering their relatively benign course, these post-COVID or post-vaccine vasculitides seem to be amenable to 0.8 to 1 mg/kg prednisolone or equivalent, which could be gradually tapered. The need for immunosuppression and the duration of steroid therapy should be determined on an individual basis. While the world still reels from the perils of a deadly pandemic, the aftermath continues to haunt. Our narrative review aims to explore the effects of COVID and the vaccine on systemic vasculitis, as well as the effect of disease and immunosuppression on the immunogenicity of the COVID vaccine.

Parkinsonism as the presenting manifestation of lupus: A case-based review.

INTRODUCTION: Neuropsychiatric manifestations in systemic lupus erythematosus (SLE) occur in about half of the patients; however, movement disorders like Parkinsonism are rare. We describe a case of SLE who presented solely with features of Parkinsonism. CASE REPORT: 50-year-old female presented with global slowing of movements and slowing of speech since 2 months. On examination, she had mask-like facies with a faint malar rash sparing the nasolabial folds, hard palate ulcer, cog-wheel rigidity, and proximal muscle weakness. Lab evaluation revealed lymphopenia, high ESR, elevated lactate dehydrogenase, creatinine phosphokinase, AST, and ALT levels. She had high anti-dsDNA levels with low complements. Urinalysis showed proteinuria and hematuria. ANA was positive at a titer of 1:320, and she had positive anti-ribosomal-P antibody. She had severe flare with a SLEDAI of 33. She was treated with pulse IV methylprednisolone followed by cyclophosphamide (NIH protocol). At 4 weeks follow-up, she had dramatic improvement in her Parkinsonian symptoms and her proximal muscle weakness. DISCUSSION: The prevalence of movement disorders in cases of neuropsychiatric SLE is very low at 0.7%, with chorea being most frequent and Parkinsonism rare. The pathogenesis is multifactorial including anti-dopaminergic antibodies or associated anti-phospholipids causing microvascular thrombosis or vasculitis of the thalamostriatal arteries or disease activity itself. As in our case, immunosuppression and optimal treatment of active lupus reverts symptoms in most cases. CONCLUSION: A high index of suspicion needs to be exercised in cases of SLE presenting with Parkinsonism as adequate immunosuppression translates to near-complete recovery.

Assessment of cutaneous disease activity in early lupus and its correlation with quality of life: a cross-sectional study.

Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease with varied dermatological manifestations that are almost universal. Overall, lupus disease has a major effect on the quality of life in these patients. We assessed the extent of cutaneous disease in early lupus and correlated it with the SLE quality-of-life (SLEQoL) index and disease activity measures. Patients diagnosed as SLE with the skin involved were recruited at the first presentation and were assessed for cutaneous and systemic disease activity using the cutaneous lupus erythematosus disease area and severity index (CLASI) and the Mexican-SLE disease activity index (Mex-SLEDAI), respectively. Quality of life was assessed with the SLEQoL tool while systemic damage was captured by the SLICC damage index. Fifty-two patients with SLE who had cutaneous involvement were enrolled (40, 76.9% females) with a median disease duration of 1 month (1-3.7). The median age was 27.5 years (IQR: 20-41). Median Mex-SLEDAI and SLICC damage index were 8(IQR: 4.5-11) and 0 (0-1), respectively. The median CLASI activity and damage scores were 3 (1-5) and 1 (0-1), respectively. Overall, there was no correlation between SLEQoL with CLASI or CLASI damage. Only the self-image domain of SLEQoL correlated with total CLASI (ρ = 0.32; p = 0.01) and CLASI-D (ρ = 0.35; p = 0.02). There was a weak correlation of CLASI with the Mexican-SLEDAI score (ρ = 0.30; p = 0.03) but not with the SLICC damage index. In this cohort of early lupus, cutaneous disease activity in lupus had a weak correlation with systemic disease. Cutaneous features did not appear to influence the quality of life except in the self-image domain.

Refractory alopecia in lupus treated with tofacitinib - a case-based review.

One of the common cutaneous symptoms of systemic lupus erythematosus (SLE) that may have major psychosocial effects in a female is diffuse alopecia. Although Janus kinase inhibitors have shown encouraging results in the treatment of SLE and of alopecia areata in recent studies, tofacitinib in treating refractory alopecia caused by SLE has been rarely documented. The Janus kinases (JAKs) are intracellular tyrosine kinases that play a significant role in the pathophysiology of SLE by participating in a wide range of inflammatory cascades. Here, we reported a 33-year-old SLE patient with long standing (3 years) refractory alopecia who took tofacitinib and observed a substantial increase in hair growth. This was sustained at 2-years follow-up even after tapering off glucocorticoids completely. In addition, we reviewed the literature to look for further evidence to support the use of JAK inhibitors for alopecia in SLE.