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Cases of systemic lupus erythematosus (SLE) following psoriatic arthritis (PsA) or vice versa are uncommon. Due to the complexity of autoimmune diseases and the rarity of such cases, comprehensive global data on the co-occurrence of these conditions are limited. Moreover, the pathophysiology concerning the coexistence of SLE and PsA has yet to be fully understood. Interestingly, the progression of both diseases appears to be significantly influenced by the key interleukin (IL) 17, particularly IL-17A. Here, we report 7 cases of SLE and PsA coexistence. In 5 of these cases, PsA occurred before the development of SLE, while in the remaining 2 cases, SLE was diagnosed before PsA. The PsA was characterized mainly by peripheral arthritis without any axial involvement, while the manifestations of SLE varied, with 3 developing systematic severe manifestations. Therapeutic challenges were posed in all cases, as treating one condition could worsen the other. Finally, we review the literature providing the current knowledge on the coexistence of these conditions. Overall, all reported cases emphasize the importance of personalized treatment and careful monitoring for patients with both SLE and PsA.
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Lichen sclerosus et atrophicus (LSA) is an inflammatory dermatosis of unknown etiology, usually affecting the genital region, with extragenital involvement being uncommon. The coexistence of LSA and morphea in the same lesion is rare. The present study aims to demonstrate that LSA and morphea might share similar pathologic processes. We present a case of a 53-year-old female patient with extragenital lesions with clinical appearance and histopathological features of both LSA and morphea. Finally, the two diseases might lie on the same disease spectrum.
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Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare multisystem necrotizing small and medium-sized vessel vasculitis with eosinophilic adult-onset asthma as part of its spectrum. Therapeutic choices include corticosteroids, immunosuppressive agents, and novel immunomodulators. Mepolizumab and benralizumab are monoclonal antibodies targeting Interleukin-5 (IL-5), which plays a leading role in every stage of production and maturation of eosinophils and are recently undergoing evaluation and administered in steroid-dependent, relapsing and/or refractory EGPA. Herein we describe the cases of three patients with a prior EGPA diagnosis, experiencing frequent asthmatic exacerbations despite oral and inhaled corticosteroid treatment (two patients) and adverse effects of corticosteroids (one patient). Two patients are under treatment with mepolizumab and one patient with benralizumab as an add-on supplemental regimen. In our case series anti-IL5 monoclonal antibodies proved efficient asthma-controlling and corticosteroid-sparing agents.
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The impact of golimumab (GLM) on remission or low disease activity (LDA) was evaluated in patients with moderate-to-severe rheumatoid arthritis (RA), progressive psoriatic arthritis (PsA), or severe axial spondyloarthritis (axSpA), who failed previous treatment for their rheumatic disease with one initial tumor necrosis factor α inhibitor (TNFi). This is a multicenter, prospective, real-world observational 18-month study, conducted in Greece. The primary endpoint, assessed at 6 months, included the proportion of patients attaining LDA and/or remission (Disease Activity Score for 28 joints based on C-reactive protein [DAS28-CRP] ≤ 3.2), minimal disease activity (MDA; MDA criteria), and moderate disease activity (Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] score 4-7), respectively. Other endpoints evaluated the persistence to GLM treatment and its impact on patients' work productivity (Work Productivity and Activity Impairment [WPAI] instrument) and quality of life (QoL; EuroQoL5 dimensions 3 levels [EQ-5D-3L] questionnaire). Descriptive statistics, the Wilcoxon signed-rank test, and Kaplan-Meier method were used for analyses. At 6 months, LDA was achieved by 46.4% of patients with RA, MDA by 57.1% of patients with PsA, and BASDAI 4-7 by 24.1% of patients with axSpA. For all study patients, persistence rates on GLM were high (85.1-93.7%) over 18 months; all WPAI domain scores and the EQ-5D-3L index score improved significantly (p < 0.001) from baseline to 18 months. GLM treatment was effective in patients with RA, PsA, or axSpA who had failed previous treatment with one TNFi and led to significant WPAI and QoL improvements. Persistence rates were high. Trial registration number and date of registration: As per the local regulations the study has been registered at the national registry for non-interventional studies https://www.dilon.sfee.gr/studiesp_d.php?meleti_id=MK8259-6995 .
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Antirreumáticos , Artrite Psoriásica , Artrite Reumatoide , Espondiloartrite Axial , Humanos , Adulto , Artrite Psoriásica/diagnóstico , Artrite Psoriásica/tratamento farmacológico , Qualidade de Vida , Estudos Prospectivos , Grécia , Resultado do Tratamento , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Antirreumáticos/uso terapêuticoRESUMO
To evaluate the effect of the phosphodiesterase 4 inhibitor apremilast in biologic-naïve patients with early peripheral PsA in terms of disease activity, clinical manifestations, patient-perceived outcomes, as well as apremilast's safety profile in routine care settings of Greece. Non-interventional, multicenter, 52-week prospective cohort study, enrolling biologic-naïve patients with early active peripheral PsA who started apremilast after intolerance or inadequate response (within the first 12 months of treatment) to an initial conventional synthetic (cs)DMARD treatment. Non-responder imputation was applied for missing data.In total, 167 consecutive patients (mean age: 52.5 years; median PsA duration: 0.9 years) were analyzed. At baseline, the median (interquartile range) clinical Disease Activity in Psoriatic Arthritis (cDAPSA) score was 22.0 (16.0-29.0), with 86.8% of patients having at least moderate (29.3% high) disease activity; 87.4% had skin psoriasis, 37.7% nail psoriasis, 30.7% enthesitis, and 12.4% dactylitis. At 16, 24, and 52 weeks, 28.7, 42.5, and 48.5% of patients, achieved ≥ 50% improvement in their baseline cDAPSA score, respectively. At week 52, 55.6, 50, and 26.8% of evaluable patients achieved complete resolution of enthesitis, dactylitis and nail psoriasis, respectively. Improvements were also observed in patient's health state assessed by the Psoriatic Arthritis Impact of Disease 12-item questionnaire, and health-related quality of life. The 52-week drug survival rate was 75%, while 13.8% of patients experienced at least one adverse drug reaction.Biologic-naïve patients with early PsA, treated with apremilast experienced significant improvements in disease activity, extra-articular manifestations and patient-centered outcomes, accompanied by a favorable tolerability profile.
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Anti-Inflamatórios não Esteroides , Artrite Psoriásica , Produtos Biológicos , Psoríase , Humanos , Pessoa de Meia-Idade , Artrite Psoriásica/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Entesopatia , Estudos Prospectivos , Psoríase/tratamento farmacológico , Qualidade de VidaRESUMO
Introduction: Patients with rheumatoid arthritis (RA) are at increased risk for serious infections. Pneumococcal vaccination is among the most important preventive measures, however, vaccine uptake is suboptimal. We explored the rate and factors associated with pneumococcal vaccination in a contemporary RA cohort. Materials and methods: Multi-center, prospective, RA cohort study in Greece. Patient and disease characteristics and influenza and pneumococcal vaccinations were documented at baseline and 3 years later. Results: One thousand six hundred and ninety-seven patients were included and 34.5% had already received at least one pneumococcal vaccine at baseline. Among 1,111 non-vaccinated patients, 40.1% received pneumococcal vaccination during follow-up, increasing the vaccine coverage to 60.8%. By multivariate analysis, positive predictors for pneumococcal vaccination included prescription of influenza vaccine (OR = 33.35, 95% CI: 18.58-59.85), history of cancer (OR = 2.35, 95% CI: 1.09-5.06), bDMARD use (OR = 1.85, 95% CI: 1.29-2.65), seropositivity (OR = 1.47, 95% CI: 1.05-2.05), and high disease activity (DAS28-ESR, OR = 1.33, 95% CI: 1.17-1.51). Male sex (OR = 0.65, 95% CI: 0.43-0.99) was a negative predictor for pneumococcal vaccination during follow-up. Discussion: Despite increasing rates of pneumococcal vaccine coverage, 40% of RA patients remain unvaccinated. Severe disease, bDMARD use, comorbidities, and more importantly flu vaccination were the most significant factors associated with pneumococcal vaccination, emphasizing the currently unmet need for cultivating a "vaccination culture" in RA patients.
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Psoriatic onycho-pachydermo periostitis (POPP) is characterized by psoriatic onychodystrophy, connective tissue thickening, and periostitis of the distal phalanges (DPs), producing a drumstick-like deformity. Our aim was to present the first case of POPP treated successfully with an IL-17 inhibitor, perform a literature review of its characteristics and treatment, and explore the possible pathogenesis. We conducted a systematic review of previously presented POPP cases. We present a patient with methotrexate (MTX)-resistant treatment POPP, who had significant resolution of symptoms and inflammatory lesions on post-treatment MRI with secukinumab 150 mg. We also identified 31 cases of POPP (27 males; mean age 44.9 years) in the literature review. There was great toe involvement in 24 cases, and distal interphalangeal (DIP) involvement in 14 cases, with frequent radiographically evident damage. Seventeen of 31 patients received systematic treatment other than biologics, mostly MTX, with no satisfactory results. Anti-TNF agents were used successfully in 5 cases, mostly after disease modifying anti-rheumatic drug (DMARD) failure. Imaging studies in nail psoriasis and DIP psoriatic arthritis have shown an anatomical link among the nail, the DP bone, and the DIP joint entheses, suggesting that POPP may be a subtype of nail disease with excessive involvement of DP tissues (nail, soft tissue, enthesis, and bone). IL-17 inhibition could be an alternative therapeutic option in DMARD-resistant cases of POPP. Conventional treatment achieves modest success, but anti-TNF agents appear to be much more effective. Based on imaging studies, POPP may be a particular subtype of nail disease.
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Artrite Psoriásica , Doenças da Unha , Periostite , Adulto , Artrite Psoriásica/complicações , Artrite Psoriásica/diagnóstico por imagem , Artrite Psoriásica/tratamento farmacológico , Humanos , Interleucina-17 , Masculino , Pessoa de Meia-Idade , Doenças da Unha/tratamento farmacológico , Periostite/diagnóstico por imagem , Periostite/tratamento farmacológico , Inibidores do Fator de Necrose TumoralRESUMO
OBJECTIVE: To study the outcome of pregnancy in patients with systemic vasculitis (SV) compared with age-, BMI- and ethnicity-matched healthy pregnant controls. METHODS: Fifty-one pregnancies in 29 SV patients were retrospectively studied. There were nine patients with granulomatosis with polyangiitis (GPA), three with eosinophilic GPA, seven with Takayasu's arteritis, two with ANCA-positive vasculitis with renal involvement, two with Behçet's disease, three with urticarial vasculitis, one with primary cerebral vasculitis, one with relapsing polychondritis and one with IgA vasculitis. BVAS and the vasculitis damage index were evaluated retrospectively. Sixty-two healthy women with 156 pregnancies matched in a 2:1 ratio for age, BMI and ethnicity formed the control group. RESULTS: Median gestational age at delivery was lower in the SV group: 36 weeks and 2 days (34-42) vs controls 40 (37-42) weeks (P < 0.03). Median birth weight in the SV group was 3.0 kg (2.0-5.2), whereas that of the controls was 3.5 (2.28-4.32) kg (P = 0.004). The median customized birth weight centile was 38.6 in the SV group and 37.2 in the control group. In the SV group, 9 patients had 13 miscarriages, 3 had pre-eclampsia, and 2 had an intrauterine death. In the control group, 20 patients had 27 miscarriages, 1 had pre-eclamptic toxaemia, and 1 had an antepartum haemorrhage. Eight patients with SV flared during pregnancy and 11 flared after delivery. CONCLUSION: Patients with SV had a lower median gestational age, but customized birth weights were similar to those of healthy women. Women with SV may flare during pregnancy and the post-partum period and may experience significant pregnancy morbidity.
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Peso ao Nascer , Idade Gestacional , Complicações Cardiovasculares na Gravidez , Resultado da Gravidez , Vasculite Sistêmica/complicações , Aborto Espontâneo/epidemiologia , Adulto , Estudos de Casos e Controles , Feminino , Hemorragia/epidemiologia , Humanos , Incidência , Pessoa de Meia-Idade , Período Pós-Parto , Pré-Eclâmpsia/epidemiologia , Gravidez , Estudos RetrospectivosRESUMO
Chronic wounds develop when the sequence of healing events are disrupted, usually in patients with underlying diseases such as diabetes mellitus, venous insufficiency, peripheral artery disease, and neuropathies and they affect most often the lower extremities. We present a 68-year-old woman with plantar ulceration, lasting for approximately 18 months, resistant to healing with conventional therapy and various modalities we used. The patient had a long history of seronegative enteropathic arthritis, Crohn's disease, secondary fibrillar amyloidosis, multiplex neuropathy, and small vessel vasculitis, the latter being the trigger event for the ulceration of her right foot. Before the decision for a final surgical intervention, we implemented a mechanical periodic stimulus using a soft toothbrush, which resulted in the gradual and complete healing of the ulcer within a period of 6 weeks. Patient's history and previous treatments are presented along with the procedures that led to the healing of the chronic wound. This report supports the idea that periodic mechanical stimulus is of great importance for the healing process and this could be the mechanism of action of some other methods that have been described in the medical literature.
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Úlcera da Perna/terapia , Modalidades de Fisioterapia , Cicatrização , Idoso , Doença Crônica , Feminino , Seguimentos , Pé , Humanos , Úlcera da Perna/diagnóstico , Estimulação Física/métodos , Índice de Gravidade de DoençaRESUMO
We report a case of acute motor and sensory neuropathy during a flare of ulcerative colitis. A 28-year-old male presented with a flare of distal ulcerative colitis despite treatment with mesalamine enemas and suppositories simultaneously with rapidly deteriorating weakness and needle sensation in both legs. Neurological assessment showed axonal sensorimotor polyneuropathy affecting mainly the lower limbs and to a lesser extent the upper limbs. Colonoscopy revealed moderately to severe active ulcerative colitis with a patchy distribution involving the rectum and the right colon. Vitamin and folic acid levels were normal. Virological, immunological and other laboratory tests were negative except for positive anti-ganglioside antibodies (anti-GM1). Ulcerative colitis and polyneuropathy improved when patient was treated with immunosuppressive therapy (corticosteroids, immunoglobulin and azathioprine). Peripheral polyneuropathy is a rare extraintestinal manifestation of ulcerative colitis and it is probably associated with an autoimmune pathogenetic mechanism.