Recent progress in systemic sclerosis-interstitial lung disease

Purpose of review Systemic sclerosis is a systemic autoimmune disorder wherein interstitial lung disease (ILD) is the major source or morbidity and mortality. Research into mechanisms of fibrosis and its intersection with autoimmunity, specifically lung fibrosis, has accelerated and been applied to autoimmune disorders such as scleroderma. This review highlights important emerging insights and treatment trials. Recent findings The important elements of this review focus on the challenges faced in identifying patients not only who develop lung disease but who are at a higher risk for progression given the heterogeneous natural history of ILD in scleroderma. Risk assessment scoring models using radiographic and physiologic parameters are highlighted and recent and ongoing clinical trials in scleroderma ILD are discussed. Summary The implications of much of this ongoing work is a potential paradigm shift in our ability to identify those patients at risk for progression, and to offer novel therapies that can limit the progression of inflammatory and fibrotic lung disease in this challenging group of patients.
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: RAYNAUD PHENOMENON, SCLERODERMA, OVERLAP SYNDROMES AND OTHER FIBROSING SYNDROMES: Edited by John Varga Source Type: research

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CONCLUSIONS: As the present study was the first investigation on the coagulation status in patients with AA, elevated D-dimer levels in alopecia areata may suggest a deficient coagulation in these patients that may contribute to an increase in the risk of thrombosis. Further studies are needed to evaluate this hypothesis using a larger sample size. PMID: 33034439 [PubMed - as supplied by publisher]
Source: Giornale Italiano di Dermatologia e Venereologia - Category: Dermatology Tags: G Ital Dermatol Venereol Source Type: research
Background: Controversy exists regarding the need for proximal fibular epiphysiodesis in conjunction with proximal tibial epiphysiodesis to prevent relative overgrowth of the fibula. The purpose of this study was to determine the incidence of relative fibular overgrowth in patients who had undergone proximal tibial epiphysiodesis with or without proximal fibular epiphysiodesis to manage leg-length discrepancy. Methods: We identified patients who had undergone proximal tibial epiphysiodesis, with or without concomitant fibular epiphysiodesis, followed to skeletal maturity, and with adequate scanograms to measure tibial...
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Leg Length Discrepancy Source Type: research
Conclusions: In this dual-center retrospective series, the single-incision triple innominate osteotomy was extremely effective for improving acetabular coverage and stabilizing unstable hips in a variety of underlying diagnoses with an acceptably low rate of complications. Level of Evidence: Level IV—case series.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Selected Topics Source Type: research
Conclusions: The authors rejected their null hypothesis. Children do have the capacity to remodel radiographically measurable sagittal plane malunion of SCHFs. Children younger than 5 years of age can remodel 100% displacement of the center of the capitellum, whereas those over 8 years of age have minimal remodeling capacity.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Upper Extremity Source Type: research
Conclusions: Distal radius fractures have a large capacity to remodel in the pediatric population. This remodeling occurs in a predictable and reliable manner, even in the coronal plane. On the basis of this study, coronal angulation was shown to remodel at a rate of at least 2 degrees per month for the first 6 months following the injury, which should likely continue at a similar rate for the first year after the injury. Repeat manipulation is not indicated in skeletally immature patients where the maximum coronal angulation is
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Upper Extremity Source Type: research
Conclusions: Intra-articular distal radius fractures in skeletally immature patients have a considerably higher rate of physeal growth arrest than extra-articular physeal fractures. Following acute management aimed at restoring and preserving anatomic physeal and articular alignment, follow-up radiographs should be obtained to evaluate for physeal arrest in skeletally immature children. Patients and families should be counseled regarding the high rate of growth disturbance and the potential need for deformity correction in the future, particularly in younger children. Level of Evidence: IV—case series.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Upper Extremity Source Type: research
Background: Chronic Monteggia lesions in children may cause pain, deformity, decreased range of motion, and neurological symptoms. Numerous surgical techniques have been advocated to reconstruct long-standing Monteggia injures in efforts to maximize long-term upper limb function. The purpose of this investigation was to assess the clinical and radiographic results of a modified surgical technique for missed Monteggia fracture-dislocations. Methods: A retrospective evaluation of 52 patients who underwent surgical reconstruction of missed Monteggia fracture-dislocations at a tertiary pediatric hospital was performed. Th...
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Upper Extremity Source Type: research
Conclusions: In this multicenter series of 125 APSCDs no injuries to the great vessels/mediastinal structures requiring intervention were identified. Although more than half of patients had evidence of extrinsic vascular compression at the time of injury, careful open reduction of acute injuries can be safely performed. Although vascular injuries following APSCD seem to be quite rare, vascular complications can be catastrophic. Treating providers should consider these data and their own institutional resources to maximize patient safety during the treatment of APSCD. Level of Evidence: Level III—therapeutic case control study.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Upper Extremity Source Type: research
Conclusions: Heavier children have worse radiographic outcomes and higher complication rates with the use of FIN for femoral shaft fractures. Additional research is needed to determine the effect of FIN material on clinical outcomes in heavier children, and the relationship between weight and other known risk factors for poor outcome in FIN, such as length stability. Level of Evidence: Level III—systematic review of level-III studies.
Source: Journal of Pediatric Orthopaedics - Category: Orthopaedics Tags: Trauma Lower Extremity Source Type: research
CONCLUSIONS: Anti-SAFB Ab is a novel CTD-related autoAb possibly associated with ILD. PMID: 27682649 [PubMed - as supplied by publisher]
Source: Journal of Autoimmunity - Category: Allergy & Immunology Authors: Tags: J Autoimmun Source Type: research
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