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.

Long-term survivors of cystic fibrosis (CF) have a dramatic increase in the risk of osteoporosis and incident fracture. The objective of this work is to characterize a CF related bone disease in a Portuguese cohort of CF patients.

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.

Systemic sclerosis (SSc) is an uncommon condition, with a wide range of manifestations, characterized by specific antibody production, vasculopathy and fibrosis of the skin and other internal organs. It is a complex disease, which is estimated to be rare in Portugal, although specific incidence data are missing. The aetiology of SSc remains unknown, but is likely to be multifactorial, involving genetic and environmental aspects. Its management is challenging and often requires a multidisciplinary approach. In 2011, we established a dedicated outpatient clinic for patients with SSc. Clinical data of every patient with a confirmed diagnosis of SSc is prospectively registered in Reuma.pt/SSc. In this manuscript, we aim to describe the general functioning of our SSc outpatient clinic, and to characterise the population of patients with SSc who are followed herein.

Psoriatic disease (Psoriasis and Psoriatic Arthritis, PsD) is a condition that affects the skin, the musculoskeletal system, and beyond, impairing patients' quality of life. A multidisciplinary approach of combined dermatology-rheumatology clinics is recommended and valuable to respond to PsD diagnosis, management, and treatment challenges. In Portugal, five Hospitals have implemented a multidisciplinary clinic for PsD assessment. This report aims to describe how these multidisciplinary clinics were developed, their characteristics, and the main obstacles to their implementation. Although the different hospitals adopted distinct functional models, a consensus respecting the minimal core set assessment for PsD in Multidisciplinary Dermatology/Rheumatology Clinics should comprise all disease manifestations and, if possible, quality of life. The main objective of these clinics is to achieve remission/minimal disease activity. Limitations to these multidisciplinary approaches are discussed, namely financial, time management, and human resources obstacles that can be a handicap in their implementation, despite the benefits of PsD integrated care.

Bone marrow edema syndrome is a rare disease with an unknown etiology, self-limited and usually associated with an indolent course, which can also generate severe pain with tremendous functional impairment. The authors present a case of a 19-year-old female patient with a progressive, non-traumatic and unrelentless pain involving both knees, requiring persistently walking aids and analgesic drugs. The imaging studies showed a bilateral distal femur and proximal tibia bone marrow edema in the magnetic resonance imaging. Finally, and after an extensive investigation without any abnormal findings, a bone marrow edema syndrome diagnosis was established, with a spontaneous regression of the clinical and imaging presentation. One year after the initial complaints the patient is fully recovered, without pain or medication, presenting an MRI showing complete regression of the initial findings. Despite the rarity of this entity, being aware of its existance and clinical manifestations is crutial to allow a proper diagnosis. The case herein presented is, to our understanding, pragmatic regarding bone marrow edema syndrome presentation and clinical course.

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.

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.

This is the first detailed description of an erosive seronegative polyarthritis in the Vogt-Koyanagi-Harada Syndrome in Portugal.

Fragility fractures cause significant mortality and morbidity. Even though there are multiple guidelines for the management of fragility fractures, european countries still report treatment rates of less than 30%. Implementation of fracture liaison services can increase this percentage by 21%. Our goal is to describe the management of osteoporosis, in patients with hip fragility fracture treated in a portuguese hospital with no internal protocols in place.

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.

Tumor necrosis factor alpha inhibitors (TNFi) are basilar treatments in a number of inflammatory rheumatic conditions and autoimmune phenomena such as de novo neuroinflammatory events were already described in these populations under TNFi. We conducted a single-center retrospective study in a cohort of rheumatic patients treated with TNFi to characterize neurological demyelinating/inflammatory disease in these patients. We report 3 cases (n= 744): all of them had spondyloarthritis, the onset of neurological manifestations occurred between 37 and 58 years old and all of them initially presented with an optic neuritis. The neurological symptoms emerged between 13 and 26 months after starting TNFi. All patients discontinued treatment with TNFi, but one resumed therapy with symptomatic worsening, having to interrupt treatment again. All patients, latter on, fulfilled multiple sclerosis (MS) McDonald criteria 1 and were diagnosed with relapsing-remitting MS. Our study support the prior view of a risk, disease-dependent or agent-dependent, although a causal relationship is yet to be enlightened.

Ankylosing spondylitis (AS) reduces spinal mobility, which results in structural and functional impairments. Pulmonary problems eventually occur in most AS patients due to interstitial lung disease or as a result of chest wall abnormalities. The aim of this study was to evaluate the effects on pulmonary functions and disease related scales of aquatic and land-based multidimensional functional mobility exercises on pulmonary functions in patients with AS.

COVID-19 changed the dynamics of all healthcare system, leading to the restructuring of inpatient teams as well as the emergency department. Scheduled surgeries were suspended, operating rooms were closed, and anesthesiologists redistributed among the various intensive care units. At the Centro Hospitalar do Baixo Vouga the number of patients admitted to the emergency department decreased to approximately 8.000 during the period of lockdown which ranged from 18th March to 1st June 2020. The aim of this study was to compare the number of patients presenting with hip fractures during the first wave of the COVID-19 pandemic with the equivalent period in 2019 and to analyze postoperative outcomes.