Acta reumatologica portuguesa最新文献

Background: Axial Spondyloarthritis (axSpA) is a chronic, inflammatory rheumatic disease that affects the axial skeleton, causing pain, stiffness, and fatigue. Genetics and environmental factors such as microbiota and microtrauma are known causes of disease susceptibility and progression. Murine models of axSpA found a decisive role for biomechanical stress as an inducer of enthesitis and new bone formation. Here, we hypothesize that muscle properties in axSpA patients are compromised and influenced by genetic background.

Objectives: To improve our current knowledge of axSpA physiopathology, we aim to characterize axial and peripheral muscle properties and identify genetic and protein biomarker that might explain such properties.

Methods: A cross-sectional study will be conducted on 48 participants aged 18-50 years old, involving patients with axSpA (according to ASAS classification criteria, symptoms duration < 10 years) and healthy controls matched by gender, age, and levels of physical activity. We will collect epidemiological and clinical data and perform a detailed, whole body and segmental, myofascial characterization (focusing on multifidus, brachioradialis and the gastrocnemius lateralis) concerning: a) Physical Properties (stiffness, tone and elasticity), assessed by MyotonPRO®; b) Strength, by a dynamometer; c) Mass, by bioimpedance; d) Performance through gait speed and 60-second sit-to-stand test; e) Histological and cellular/ molecular characterization through ultrasound-guided biopsies of multifidus muscle; f) Magnetic Resonance Imaging (MRI) characterization of paravertebral muscles. Furthermore, we will perform an integrated transcriptomics and proteomics analysis of peripheral blood samples.

Discussion: The innovative and multidisciplinary approaches of this project rely on the elucidation of myofascial physical properties in axSpA and also on the establishment of a biological signature that relates to specific muscle properties. This hitherto unstudied link between gene/protein signatures and muscle properties may enhance our understanding of axSpA physiopathology and reveal new and useful diagnostic and therapeutic targets.

Granulomatous with polyangiitis (GPA) is a necrotizing granulomatous vasculitis that mostly affects small-sized vessels. The disease can affect many organs, although renal and respiratory tract involvement are the most frequent and distinguishing features. Musculoskeletal manifestations have been reported in about 50% of patients and can occur as myalgia, oligoarthralgia/arthritis of large joints or polyarthralgia/arthritis of small joints. Infrequently musculoskeletal symptoms can be the first disease manifestation, and in this clinical scenario GPA diagnosis might be delayed or mistaken by other rheumatic diseases. The authors describe three patients with musculoskeletal symptoms as earliest GPA manifestations, illustrating the clinical challenge.

Systemic lupus erythematosus (SLE) is an autoimmune disease with potential multisystemic involvement. Mesenteric panniculitis (MP) has been described as a rare feature in patients with SLE. The authors present a case of a 26 years old patient with previous diagnosis of SLE presenting with abdominal pain and distension and a peri-umbilical mass. Imagological findings were compatible with MP and ganglion biopsy revealed inflammatory pattern. Corticosteroid therapy was initiated with a resolution of pain after 6 months of treatment, with image reevaluation showing improvement of previous findings.

Objective: The classification and/or diagnosis of Primary Sjögren's Syndrome (PSS) requires a multidimensional approach. Although age and the duration of sicca symptoms can affect the clinical, serological and histological features found at initial evaluation, these are not considered when using classification criteria as a guide for PSS diagnosis. Our study aimed to explore if there is any relationship between the duration of symptoms and clinical, histopathological and serological findings.

Methods: An observational, retrospective study was performed. All the evaluated subjects were part of the "sicca cohort". Patients' clinical, serological and histological characteristics were assessed according to the duration of symptoms. A Receiving Operator Characteristic (ROC) curve was performed to establish the duration of symptoms (months) that predicted a PSS diagnosis. Binary regression models and odds ratios were used to evaluate the association between the duration of symptoms and the clinical, serological, and histopathological profiles.

Results: One hundred and sixteen patients were included; 97(83.62%) fulfilled PSS criteria. Of the 116 patients, thirty-six (31.03%) had < 15 months presenting with sicca symptoms when receiving a diagnostic approach. A duration of symptoms >15 months was associated with an altered Schirmer test (OR 2.76; 95% CI 1.15-6.61, P=0.02), low salivary flow rate (OR 3.5; 95% CI 1.34-9.13, P=0.01), ≥1 foci score (OR 1.21; 95% CI 1-1.45, P=0.04), ocular (OR 7.8; 95% CI 1.49-40.81, P=0.02) and severe oral symptoms (OR 2.61; 95% CI 1.16-5.87, P=0.02).

Conclusion: The time of evolution of symptoms plays a fundamental role in the clinical, histological and serological profiles in PSS.

Granulocyte colony-stimulating factor (G-CSF) is increasingly being used to prevent febrile neutropenia associated with chemotherapy. Large-vessel vasculitis (LVV) has been recognized as a rare side effect of G-CSF treatment. We report a case of G-CSF associated LVV in a patient with breast cancer. While clear pathogenic mechanisms remain unknown, G-CSF may cause vasculitis due to inflammatory cytokines production. This adverse reaction should be recognized in patients with suggestive symptoms following the administration of pegfilgrastim. A 56-year-old woman with luminal B breast cancer who had undergone surgery and adjuvant chemotherapy, initially with paclitaxel, was started on a doxorubicin plus cyclophosphamide protocol, followed by supportive use of long-acting G-CSF pegfilgrastim. Following the administration of pegfilgrastim, the patient developed intermittent fever and was given empiric antibiotics in the outpatient setting with no improvement. There were no signs of cancer progression, and the contrast-enhanced CT scan highlighted wall thickening of the aortic arch and the proximal segment of the subclavian artery, which was not present in previous imaging studies. The patient was diagnosed with LVV, and a differential diagnosis was performed to rule out paraneoplastic setting, immune-mediated diseases, infection or other drug-induced vasculitis. Treatment with steroids was initiated and tapered with significant improvement and resolution of the radiological signs of aortitis.