sclerodermia

The updated S2k guideline deals with the diagnosis and therapy of localized scleroderma (LoS). LoS represents a spectrum of sclerotic skin diseases in which, depending on the subtype and localisation, structures such as adipose tissue, muscles, joints, and bones may also be affected. Involvement of internal organs or progression to systemic sclerosis does not occur. LoS can be classified into four main forms: limited, generalized, linear, and mixed forms, with some additional subtypes. For cases of limited skin involvement, the guideline primarily recommends therapy with topical corticosteroids...

Many surgical solutions for knee flexiondeformity in the pediatric population alter the anatomical bony alignment in the distal femur. Posterior knee capsule release has been presented as an alternative surgical procedurethat maintains the anatomical shape of relevant bones while solving the issue of knee flexion contracture. The aim of this study is to assess the results of a double-incision posteriorknee capsulotomy release performed on pediatric patients with neuromuscular or congenital severe knee flexion deformity...

OBJECTIVE: To investigate the patient- and caregiver-reported impact of systemic sclerosis (SSc) manifestations (hand/feet/joint involvement and pulmonary complications) on the diagnostic and therapeutic journey, working productivity, and social life. METHODS: Two questionnaires (one for the patients, n  = 260 and one for the caregivers, n  = 47) were designed in collaboration with the patients' association Gruppo Italiano per la Lotta alla Sclerodermia (GILS)...

The purpose of the study is to examine in depth and analyze renal, hepatic and immune function indices in patients with Duchenne muscular dystrophy. We analyzed the follow up clinical and laboratory data of Duchenne muscular dystrophy in 32 patients. The patients underwent a standardized examination, involving studying the medical case history, general clinical data, determining Sheldon's somatotype and the constitutional type, the detailed neurological status examination, testing a personality type, laboratory and instrumental examinations...

The 6-minute walk test (6MWT) is a standardised, feasible and reliable measure of sub-maximal exercise capacity that has never been fully validated in systemic sclerosis (SSc). A variety of data suggest that many non-pulmonary aspects of SSc contribute to the test results, thus blunting the ability of the 6MWT to measure changes in lung function. Sources of variability are a training effect, technician experience, subject encouragement, medication, other activities on day of testing, deconditioning and the effects of musculoskeletal conditions and pain...

Hand functioning is often impaired in patients with Systemic sclerosis. Neuromuscular Taping is a novel application of tape able to improve functioning. The aim of this study was to evaluate the possible role of this application in the hand functionality of patients with Systemic sclerosis. Women with a diagnosis of SSc has been recruited and evaluated using different scales before and immediately after NMT application and after one, three and six months. Fifty-three women has been evaluated and Cochin Hand Functional Disability scale, Hand Mobility in Sclerodermia, Modified Rodnan Skin Score and Dreiser Algo - Functional Index scores showed statistical significant differences during all the period; moreover a reduction of pain and Raynaud Phenomenon's and an improvement of finger flexion has been observed...

PURPOSE OF REVIEW: Inflammatory myopathies are rare diseases. Their diagnosis criteria are historically based on their clinical phenotype (topography of the muscle weakness, presence of skin lesions and/or of extra-skin/muscle signs) and the presence of inflammatory infiltrates on muscle biopsy. However, the recent discovery of different myositis-specific antibodies (MSA) or myositis-associated antibodies (MAA) permitted to revisit these old classifications. This review covers recent findings in clinical and pathological phenotypes regarding prognosis, associated cancer and response to the treatment based on MSA/MAA categorization...

By the manometric method the esophageal motility in 14 patients with sclerodermia was studied. All patients presented an esophageal motor dysfunction characterized by the decrease in amplitude of the spohageal contractions, presence of aperistaltic contractions, decrease of basal pressure of the lower esophageal sphincter and its incomplete relaxation at deglutition. These esophageal motor disturbances may appear in association or separately in the same patient. The pathogenesis of the esophageal motor dysfunction in sclerodermia is not yet fully understood...

AIMS: Pulmonary veno-occlusive disease (PVOD) is a rare condition of pulmonary arterial hypertension (PAH), in which post-capillary veins are affected. Since the therapeutic approach in PVOD differs from other forms of PAH, it is crucial to establish the diagnosis. Due to the fact that affected patients are often hemodynamically unstable, minimal invasive procedures are necessary for the diagnostic work-up. Chronic alveolar haemorrhage has been observed during bronchoalveolar lavage in PVOD cases...

Scleroderma or systemic sclerosis (SSc) is an autoimmune disease of the connective tissue, characterized by vascular abnormalities and progressive fibrosis of the skin and internal organs. Kidney, esophagus, heart and lung are most frequently involved. According to the extensive skin involvement and the internal organ injury, scleroderma is classified in limited and diffuse forms. Vascular injury is considered the first event in the pathogenesis of scleroderma. Vasculopathy primarily affects the microcirculation and the small vessels decreasing blood flow that results in chronic ischemia...

Pulmonary hypertension may be associated with many clinical conditions, including connective tissue diseases. We present a case of a patient with sclerodermia and chronic myeloid leukemia, who developed severe symptoms of pulmonary hypertension. Atypical clinical course of pulmonary hypertension, including complete remission of clinical symptoms and hemodynamic improvement provoked critical approach to the etiology of pulmonary hypertension. Taking into account the temporal coincidence with the use of dasatinib (a tyrosine kinase inhibitor) and a few case reports in the literature, it appears that reversible pulmonary hypertension in our patient was associated with the use of dasatinib...

Necrotizing myopathies (MN) are defined by a specific histological pattern. They are characterized by a predominant muscle fibre necrosis and regeneration but with little or no associated inflammation. This histological pattern is observed in acquired myopathy but also in muscular dystrophy. Acquired NM can be secondary to drugs or toxics, and if not, autoimmune mechanisms have to be suspected. Necrotizing autoimmune myopathy is recognized as a subgroup of idiopathic inflammatory myopathies, different from other myositides...

OBJECTIVES: There is currently no consensus on best practice in systemic sclerosis (SSc). To determine if variability in treatment and investigations exists, practices among Canadian Sclerodermia Research Group (CSRG) centres were compared. METHODS: Prospective clinical and demographic data from adult SSc patients are collected annually from 15 CSRG treatment centres. Laboratory parameters, self-reported socio-demographic questionnaires, current and past medications and disease outcome measures are recorded...

Exhaled breath condensate (EBC) has been increasingly used as a new and non-invasive method to study airway inflammation. In this study we have compared the concentrations of lipid mediators in EBC with concentrations in bronchoalveolar lavage fluid (BALF). We included 37 patients undergoing bronchoscopy (12 sarcoidosis, 12 COPD, 6 lung cancer, 5 chronic cough, 1 Wegener's granulomatosis, 1 sclerodermia). Patients were not allowed to have exacerbation or any change in concomitant medication for at least 4 weeks prior to the study...

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