App-based supplemental exercise in rehabilitation, adherence, and effect on outcomes: a randomized controlled trial.

Question: To determine the uptake of an app-based supplemental exercise programme in a rehabilitation setting and the effect of such a programme on length of stay and function compared to usual care physiotherapy.

Design: Randomized controlled trial with random allocation and assessor blinding.

Participants: A total of 144 individuals with mixed diagnoses (orthopaedic, neurological, reconditioning) admitted for inpatient sub-acute rehabilitation.

Nailfold capillaroscopy and autoimmune connective tissue diseases in patients from a Portuguese nailfold capillaroscopy clinic.

Raynaud's phenomenon (RP) is frequent in autoimmune connective tissue diseases (AICTD) and its approach includes nailfold capillaroscopy (NFC), as it is a non-invasive technique that permits direct visualization of the microcirculation. The aim of this study is to analyze and establish clinical correlations between NFC findings and particular aspects of autoimmune disorders. This is a retrospective study.

App-based supplemental exercise during inpatient orthopaedic rehabilitation increases activity levels: a pilot randomised control trial.

Background: There is a known positive relationship between time in therapy and therapy outcomes. Effective rehabilitation should therefore include larger doses of therapy. However, individuals participating in inpatient rehabilitation have low levels of activity throughout the day.

Systemic lupus erythematosus: state of the art on clinical practice guidelines.

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-three original guidelines published between 2004 and 2017 were identified.

Clinical Presentation and Long-Term Outcomes of Systemic Sclerosis Portuguese Patients from a Single Centre Cohort: A EUSTAR Registration Initiative.

Introduction: Systemic sclerosis is a complex disorder that requires systematic screening. Our objective is to report the European Scleroderma Trials and Research group centre affiliation and its impact in our clinical practice.

Material And Methods: The European Scleroderma Trials and Research group affiliation process, database update and current patient evaluation, with respect to demographic and clinical features.

Fatal CTLA-4 heterozygosity with autoimmunity and recurrent infections: a de novo mutation.

Primary immunodeficiency disorders are rarely diagnosed in adults but must be considered in the differential diagnosis of combined recurrent infections and autoimmune disease. We describe a patient with CTLA-4 haploinsufficiency and an abnormal regulatory T-cell phenotype. Unusually, infections were more severe than autoimmunity, illustrating therapeutic challenges in disease course.

Systemic mastocytosis - a diagnostic challenge.

Mastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally.

Hepatosplenic T-cell lymphoma: a rare cause of hepatosplenomegaly.

We report a case of a man presenting with an unexplained fever, pancytopenia and hepatosplenomegaly without lymphadenopathy. Bone marrow flow cytometry strongly suggested hepatosplenic γδ T-cell lymphoma and infiltration of bone marrow samples by pathological T-lymphocytes confirmed the diagnosis. Despite chemotherapy the patient died 1 year after diagnosis.

Eosinophilic enteritis: a rare cause of diarrhoea.

We report a case of a healthy young man presenting with 1-week history of diarrhoea, acute abdominal pain and weight loss. Laboratory investigation showed very high peripheral eosinophils levels. After exclusion of the other causes of eosinophilia, a histological bowel sample analysis revealed marked eosinophilic infiltration of a small bowel mucosal layer which confirmed the suspicion of eosinophilic enteritis.