Can microvascular damage predict disease severity in patients with systemic sclerosis?

Introduction: Systemic sclerosis (SSc) is characterized by progressive fibrosis of the skin and internal organs, microvascular damage and cellular and humoral immunity abnormalities. Microvascular damage can be easily detected through nailfold videocapillaroscopy (NVC).

Materials And Methods: A retrospective study of patients with SSc and a NVC performed within the first 6 months after diagnosis was conducted.

Assessment of calcinosis in Portuguese patients with systemic sclerosis - a multicenter study.

Introduction/objectives: The study aims to define the clinical and subclinical calcinosis prevalence, the sensitivity of radiographed site and clinical method for its diagnosis, and the phenotype of Portuguese systemic sclerosis (SSc) patients with calcinosis.

Method: A cross-sectional multicenter study was conducted with SSc patients fulfilling Leroy/Medsger 2001 or ACR/EULAR 2013 classification criteria, registered in the Reuma.pt.

Association of Anti-Saccharomyces cerevisiae antibodies with clinical phenotype in spondyloarthritis patients.

Introduction: The association between spondyloarthritis (SpA) and inflammatory bowel disease (IBD) has been shown in many studies. More recently, with the hypothesis that increased gut inflammation is of etiopathogenic importance in the development of SpA, evaluation of anti-Saccharomyces cerevisiae antibodies (ASCA) has gained increasing relevance.

Objective: To study the status and frequency of ASCA in SpA patients and the association of these biomarkers with the clinical profile.

Happiness, quality of life and their determinants among people with systemic sclerosis: a structural equation modelling approach.

Background: Patients' objectives and experiences must be core to the study and management of chronic diseases, such as SSc. Although patient-reported outcomes are attracting increasing attention, evaluation of the impact of disease on the overall subjective well-being, equivalent to 'happiness', is remarkably lacking.

Objectives: To examine the determinants of happiness and quality of life in patients with SSc, with emphasis on disease features and personality traits.

Effectiveness of switching between TNF inhibitors in patients with axial spondyloarthritis: is the reason to switch relevant?

Background: To investigate whether the reason to discontinue the first TNF inhibitor (TNFi) affects the response to the second TNFi in axial spondyloarthritis (axSpA).

Methods: Patients with axSpA from the Rheumatic Diseases Portuguese Register (ReumaPt), who discontinued their first TNFi and started the second TNFi between June 2008 and May 2018, were included. Response was assessed by the Ankylosing Spondylitis Disease Activity Score (ASDAS) clinically important improvement (ASDAS-CII), major important improvement (ASDAS-MI), low disease activity (ASDAS-LDA), and inactive disease (ASDAS-ID).

Eligibility criteria for biologic disease-modifying antirheumatic drugs in axial spondyloarthritis: going beyond BASDAI.

Objectives: To compare definitions of high disease activity of the Ankylosing Spondylitis Disease Activity Score (ASDAS) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in selecting patients for treatment with biologic disease-modifying antirheumatic drugs (bDMARDs).

Methods: Patients from Rheumatic Diseases Portuguese Register (Reuma.pt) with a clinical diagnosis of axial spondyloarthritis (axSpA) were included.

Chondrosarcoma as inaugural manifestation of monostotic Paget's disease of Bone.

Sarcomatous degeneration is one of the serious and rare complications of Paget's disease of bone. Osteosarcoma is the most common secondary tumour, while other variants such as chondrosarcoma are extremely uncommon. We describe a unique case of Paget's chondrosarcoma of the pelvis in an elderly female patient, with no previous established diagnosis of osteitis deformans.

Osteopoikilosis: case series from Portuguese Rheumatology Centers.

Osteopoikilosis (OPK) is a rare, hereditary, usually asymptomatic disease characterized by the presence of multiple, well-defined sclerotic lesions distributed in peri-articular locations, frequently diagnosed as an incidental finding. Differential diagnosis with osteoblastic metastases is fundamental. This article reports six cases of OPK diagnosed in Portuguese Rheumatology Centers.

Haemophagocytic syndrome in Systemic Lupus Erythematosus - clues to an early diagnosis.

Macrophage activation syndrome (MAS) is a rare life-threatening condition that involves excessive activation of inflammatory cells and overproduction of different cytokines. It is characterized by persistent fever, hepatosplenomegaly, cytopenias and coagulopathy. Other prominent features are hyperferritinemia and findings of activated macrophages in haemopoietic organs, often associated with multi-organ impairment.

Silent acute myocarditis in eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis is a rare multisystemic disorder, characterized by necrotizing vasculitis affecting small to medium-sized vessels, associated with asthma and eosinophilia. Cardiac involvement is the most important predictor of mortality and it seems to be more frequent in anti-neutrophil cytoplasmic antibodies-negative patients. Cardiomyopathy and congestive heart failure can occur but a significant proportion of patients are asymptomatic.