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.

Tofacitinib in Refractory Scleritis: A Case Series.

Tofacitinib, a Janus kinase inhibitor, has been recently investigated as a potential therapy for refractory scleritis. Despite treatment with systemic immunosuppressive agents, scleritis is refractory to conventional therapy in a significant number of patients. Hereby, we report the use of tofacitinib as a steroid-sparing immunomodulatory agent in three patients with refractory scleritis who were either recalcitrant or intolerant to conventional therapy.

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.

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.

Restless leg syndrome in rheumatic conditions: Its prevalence and risk factors, a meta-analysis.

INTRODUCTION: Restless leg syndrome (RLS) is a neurological disorder characterized by an uncontrollable desire to move legs along with abnormal sensations, particularly at night, which can lead to sleep disturbance. RLS may mimic rheumatic diseases or can be associated with them, hence their identification and treatment are important to improve sleep quality and overall quality of life in rheumatic diseases. METHODS: We conducted a search of the PubMed, SCOPUS, and EMBASE databases to identify studies reporting a prevalence of RLS in patients with rheumatic disease. Two authors independently screened, selected, and extracted the data. Heterogeneity was assessed using I2 statistics and random effect method of the meta-analysis was used to synthesize the results. RESULTS: Out of 273 unique records, 17 eligible studies including 2406 rheumatic patients were identified. RLS prevalence (95% CI) among patients of rheumatoid arthritis, systemic lupus erythematosus, osteoarthritis, fibromyalgia and ankylosing spondylitis are found to be 26.6% (18.6 34.6); 32.5% (23.1-41.9), 4.4% (2.0-6.8), 38.1% (31.3-45.0) and 30.8% (23.48-39.16) respectively. RLS prevalence was similar for males and females. CONCLUSION: Our study indicates a high prevalence of RLS in patients with rheumatic diseases. Early detection and treatment of RLS in patients with rheumatic conditions could be beneficial in improving their overall health and quality of life.

Trigeminal Neuralgia as an Initial Presentation of Systemic Autoimmune Diseases: A Case Series.

Background: Trigeminal neuralgia, also known as tic doloureaux, refers to a sudden onset of unilateral or bilateral facial numbness with or without pain or paraesthesia. Trigeminal neuralgia is rare in connective tissue diseases (CTD); however, it is the most common neurologic manifestation of mixed connective tissue (MCTD) and maybe the only presenting symptom in various CTDs. Patients and Methods: Here, we describe a series of four cases of various autoimmune connective tissue diseases where trigeminal neuralgia was the presenting complaint. The first 2 cases were MCTD patients, and the 3rd case was a patient with diffuse cutaneous systemic sclerosis (SSc) and the 4th case had overlap syndrome (primary Sjogren's syndrome with SSc). The relevant literature describing trigeminal neuralgia in CTD was reviewed. The authors performed a systematic search of patients with Trigeminal neuralgia and Connective tissue diseases in PubMed, Scopus from January 1970 until July 2022. Results: All our cases had trigeminal neuralgia as presenting symptom which suggests that trigeminal neuralgia may be one of the presenting symptoms of several systemic autoimmune diseases that often cause a significant delay in diagnosis and treatment. We selected 15 records for the literature review. Conclusion: Any patient who presents with trigeminal neuralgia which responds poorly to medical management should be properly examined for underlying primary systemic autoimmune diseases.

Successful use of tofacitinib in reactive arthritis refractory to conventional therapies - a case series.

Reactive arthritis is an immune-mediated aseptic arthritis resulting from either genitourinary or gastrointestinal tract in a genetically susceptible host. It commonly presents as oligoarthritis of the lower limbs with or without extra-articular features such as urethritis and non-purulent conjunctivitis. Therapies include non steroidal anti inflammatory drugs (NSAIDs), conventional disease modifying anti-rheumatic drugs (DMARDs) and, rarely, biologics in severe cases. We report the successful use of tofacitinib in four cases of reactive arthritis who failed to respond to conventional therapies.

Secukinumab therapy in reactive arthritis: Report of two cases.

Reactive arthritis (ReA) is an immune-mediated aseptic synovitis resulting either from genitourinary or gastrointestinal tract, commonly presenting as oligoarthritis of the lower limbs and rarely urethritis and conjunctivitis. The treatment options include nonsteroidal anti-inflammatory drugs, conventional disease-modifying antirheumatic drugs, and biologics in severe cases. We report successful use of secukinumab in two cases of chronic severe ReA who initially failed to treatment with tumour necrosis factor inhibitor.

A rare case of atypical Cogan's syndrome presenting as encephalitis.

Cogan's syndrome (CS) is a rare autoimmune vasculitis of unknown aetiology characterised by non-syphilitic interstitial keratitis, audiovestibular symptoms, sometimes systemic symptoms, and multi-organ involvement. Atypical CS has other ocular features, such as scleritis, episcelritis, retinitis, and optic neuritis. Diagnosis of CS is purely clinical without a confirmatory test. Hereby, we report a case of atypical CS presenting with features of encephalitis who was treated successfully with intravenous pulse methylprednisolone with cyclophosphamide. It is important to consider CS in the differential diagnosis of encephalitis with ocular and vestibular symptoms in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.

Unusual Cause of Vision Loss in a Case of Lupus.

Lupus can affect vision in various ways, commonly due to vasculitic retinopathy, and can also result from ischemic optic neuropathy secondary to antiphospholipid syndrome. Anaemic retinopathy is most likely to occur in patients with severe anaemia or associated with thrombocytopenia. Causes of decreased visual acuity in anaemia include haemorrhages involving the macula, macular oedema, optic disc oedema and ischemic optic neuropathy. We herein describe an unusual cause of loss of vision in a lupus patient associated with severe anaemia and thrombocytopenia. Ocular examination was suggestive of anaemic retinopathy. Her vision improved with concomitant resolution of retinal findings in 1 month after treatment for lupus with anaemia correction. Hence, even though vision loss in lupus is dreadful, retinal changes in fundus examination should be carefully looked at to rule out anaemic retinopathy, as it is almost always reversible with correction of anaemia unlike lupus retinopathy.

Musculoskeletal Disorders in Chronic Obstructive Airway Diseases: A Neglected Clinical Entity.

Chronic obstructive pulmonary disease (COPD) is a lung disease that can affect various extra-pulmonary organs; one being the musculoskeletal system. Skeletal muscle dysfunction and osteoporosis are two important musculoskeletal disorders that have an impact on the quality of life in COPD patients in terms of morbidity and mortality. Treatment related adverse effects of COPD such as steroid-induced myopathy and osteoporosis are well recognised. Other comorbidities like sarcopenia, cardiovascular disease, metabolic diseases (diabetes mellitus, obesity, and thyroid diseases), chronic kidney disease, sleep apnoea, anaemia, and depression are also noted, which can contribute to impaired health status, increased healthcare utilisation, and even mortality. As well, it has been shown that autoimmunity and autoimmune rheumatic diseases (AIRDs) are linked to COPD. In this mini-review, we intend to give an overview of different types of musculoskeletal disorders associated with COPD.

Autoimmune Rheumatic Diseases Masquerading as Psychiatric Disorders: A Case Series.

BACKGROUND: Neurological involvement has been found in many autoimmune diseases, with psychiatric abnormalities such as anxiety, depression, psychosis, and cognitive dysfunction. PATIENTS AND METHODS: Here, we describe a series of 5 consecutive cases of autoimmune diseases presenting with psychiatric symptoms as the predominant manifestation. RESULT: Our case series suggests that psychiatric symptoms, mainly depression, can be one of the presenting symptoms of several autoimmune diseases that often cause a significant delay in diagnosis. CONCLUSION: Any patient, particularly females, with a newly detected psychiatric disorder that responds poorly to medical management should be properly examined for underlying primary systemic autoimmune diseases.