scholarly journals AB0855 FIBROUS ARTHROPATHY AS THE KEY FEATURE OF JUVENILE SCLERODERMA - CASE REPORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1451.2-1451
Author(s):  
D. Dibrov ◽  
M. Starovoytova ◽  
O. Desinova
Keyword(s):  
Systemic Sclerosis ◽  
Morphological Changes ◽  
Rapid Development ◽  
Subcutaneous Fat ◽  
Connective Tissue Diseases ◽  
Collagen Fibers ◽  
Primary Amyloidosis ◽  
Juvenile Onset ◽  
Immunological Disorders ◽  
The Uk

Background:Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction, immunological disorders, and excessive synthesis of collagen and its deposition in various tissues and organs. The juvenile onset SSc before the age of 16 is very rare, with annual incidence of 0.27-0.5 cases per million children according to English and Finnish authors [1,2].Objectives:To present a clinical case of juvenile onset SSc, manifesting from the childhood predominantly with fibrous contractures.Methods:Patient K., 30 yo. Clinical presentation on admission to the Institute of Rheumatology in September 2020: thickening of the trunk and limbs skin (mRSS 10 scores), pronounced induration of subcutaneous tissues and muscles; contractures of the elbow, shoulder, hip and knee joints, short stature (height 142 cm) with proportional shortening of the limbs. ANA (HEp-2) 1/320, a-Scl-70, a-RNP-70 and ACA tests were negative. Ultrasonography revealed left-sided coxitis, esophagogastroduodenoscopy - Barrett’s esophagus. Chest CT, echocardiography, electrocardiography and capillaroscopy yielded no specific findings.The patient has been ill since the age of 3, when SSc manifested with skin thickening, “dry” arthritis and rapid development of contractures of the large joints. Thorough diagnostic elaboration ruled out such potential causes as phenylketonuria, glycogenosis, mucopolysaccharidoses, primary amyloidosis, and porphyria. Histological findings (2007) of a biopsied skin specimen containing subcutaneous fat and muscle tissue included focal vacuolization of keratinocytes, poor perivascular lymphocytic and histiocytic infiltration, fibrosis and hyalinosis of collagen fibers of varying intensity in the in mid- and deep dermis, infiltration of collagen fibers by fibroblasts, skin appendages atrophy – all of them representing a pattern of morphological changes characteristic of SSc. Therapeutic regimens including prednisone at 5-15 mg/day and D-penicillamine were ineffective.Results:In this case, in view of fibrotic arthropathy, a differential diagnosis was made with deep morphea and stiff skin syndrome. Visceral involvement, immunological disorders and biopsy findings substantiated a diagnosis of juvenile onset SSc. Oral MTX was initiated at 15 mg to target skin lesion and osteoarticular symptoms.Conclusion:Predominance of fibrotic arthropathy in presented case caused difficulties in establishing SSc diagnosis, as this patient did not have such inherent features as the Raynaud’s phenomenon, interstitial lung disease or pulmonary hypertension. Juvenile onset SSc manifesting before the age of 16 has its own clinical features, usually persisting through the adulthood, and therefore, such one-of-a-kind appearances of juvenile onset SSc should not be missed or misinterpreted.References:[1]Herrick AL, Ennis H, Bhushan M et al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res. 2010 Feb;62(2):213-8. doi: 10.1002/acr.20070.[2]Pelkonen PM, Jalanko HJ, Lantto RK et al. Incidence of systemic connective tissue diseases in children: a nationwide prospective study in Finland. J Rheumatol. 1994 Nov;21(11):2143-6Disclosure of Interests:None declared

Qualitative and quantitative assessment of nailfold capillaries by capillaroscopy in healthy volunteers

VASA ◽  
2012 ◽  
Vol 41 (1) ◽  
pp. 19-26 ◽  
Author(s):  
Hoerth ◽  
Kundi ◽  
Katzenschlager ◽  
Hirschl
Keyword(s):  
Scoring System ◽  
Morphological Changes ◽  
Connective Tissue Diseases ◽  
Capillary Density ◽  
Diagnostic Value ◽  
Healthy Individuals ◽  
Extreme Position ◽  
Qualitative And Quantitative ◽  
Diffuse Loss ◽  
Density Results

Background: Nailfold capillaroscopy (NVC) is a diagnostic tool particularly useful in the differential diagnosis of rheumatic and connective tissue diseases. Although successfully applied since many years, little is known about prevalence and distribution of NVC changes in healthy individuals. Probands and methods: NVC was performed in 120 individuals (57 men and 63 women; age 18 to 70 years) randomly selected according to predefined age and sex strata. Diseases associated with NVC changes were excluded. The nailfolds of eight fingers were assessed according to standardized procedures. A scoring system was developed based on the distribution of the number of morphologically deviating capillaries, microhaemorrhages, and capillary density. Results: Only 18 individuals (15 %) had no deviation in morphology, haemorrhages, or capillary density on any finger. Overall 67 % had morphological changes, 48 % had microhaemorrhages, and 40 % of volunteers below 40 years of age and 18 % above age 40 had less than 8 capillaries/mm. Among morphological changes tortous (43 %), ramified (47 %), and bushy capillaries (27 %) were the most frequently altered capillary types. A semiquantitative scoring system was developed in such a way that a score above 1 indicates an extreme position (above the 90th percentile) in the distribution of scores among healthy individuals. Conclusions: Altered capillaries occur frequently among healthy individuals and should be interpreted as normal unless a suspicious increase in their frequency is determined by reference to the scoring system. Megacapillaries and diffuse loss of capillaries were not found and seem to be of specific diagnostic value.

scholarly journals AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants

2021 ◽  
pp. 239719832110043
Author(s):  
Miguel Angel Garcés Villalá ◽  
Carolina Zorrilla Albert
Keyword(s):  
Systemic Sclerosis ◽  
Bone Loss ◽  
Dental Implants ◽  
Social Life ◽  
Connective Tissue Diseases ◽  
The Body ◽  
Calcinosis Cutis ◽  
Complex Disorders ◽  
Standardized Parameters

Introduction: Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw. Case Description: A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up. Conclusion: The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis.

scholarly journals Brief Report: HLA-DRB1, DQA1, and DQB1 in Juvenile-Onset Systemic Sclerosis

2016 ◽  
Vol 68 (11) ◽  
pp. 2772-2777 ◽  
Author(s):  
Anne M. Stevens ◽  
Sami B. Kanaan ◽  
Kathryn S. Torok ◽  
Thomas A. Medsger ◽  
Maureen D. Mayes ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Juvenile Onset

SAT0521 Juvenile Onset Connective Tissue Diseases in Norway – a Hospital Based Registry

2015 ◽  
Vol 74 (Suppl 2) ◽  
pp. 848.3-848
Author(s):  
T.M. Ingebrigtsen ◽  
T. Garen ◽  
B. Flatø ◽  
H. Sanner
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Juvenile Onset

scholarly journals Knee myopericytoma-case report and literature review

2020 ◽  
Vol 12 (6) ◽  
pp. 127-128
Author(s):  
Susana Rodrigues ◽  
Catrine Ferreira ◽  
Tiago Coelho ◽  
Diogo Gaspar ◽  
Jean Fallah ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Case Report ◽  
Systemic Sclerosis ◽  
Middle Age ◽  
Subcutaneous Tissue ◽  
Connective Tissue Diseases ◽  
Systemic Lupus Erythematous ◽  
Systemic Lupus ◽  
Calcinosis Cutis ◽  
Substantial Morbidity

Calcinosis cutis is a rare disorder characterized by of deposition of insoluble calcium salts in the skin and subcutaneous tissue. Five subtypes of calcinosis cutis are described: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.1 Of these subtypes, dystrophic calcinosis (DC) is the most common, and it is the most frequently seen in association with underlying autoimmune connective tissue diseases.2 Dermatomyositis, systemic sclerosis and less commonly systemic lupus erythematous were described to be complicated by DC. However, DC associated with rheumatoid arthritis (RA) is extremely rare.2 The condition causes substantial morbidity and is associated with pain and limitation of movement when the process involves areas close to joints or when ulceration occurs.2 We report a middle age Sudanese woman with good controlled RA who developed dystrophic calcinosis cutis.

scholarly journals Radionuclide-Based Imaging of Breast Cancer: State of the Art

Cancers ◽  
2021 ◽  
Vol 13 (21) ◽  
pp. 5459
Author(s):  
Huiling Li ◽  
Zhen Liu ◽  
Lujie Yuan ◽  
Kevin Fan ◽  
Yongxue Zhang ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Molecular Imaging ◽  
Single Photon ◽  
Morphological Changes ◽  
Imaging Techniques ◽  
Rapid Development ◽  
Photon Emission ◽  
Emission Computed Tomography ◽  
Biological Processes ◽  
Functional Perspective

Breast cancer is a malignant tumor that can affect women worldwide and endanger their health and wellbeing. Early detection of breast cancer can significantly improve the prognosis and survival rate of patients, but with traditional anatomical imagine methods, it is difficult to detect lesions before morphological changes occur. Radionuclide-based molecular imaging based on positron emission tomography (PET) and single-photon emission computed tomography (SPECT) displays its advantages for detecting breast cancer from a functional perspective. Radionuclide labeling of small metabolic compounds can be used for imaging biological processes, while radionuclide labeling of ligands/antibodies can be used for imaging receptors. Noninvasive visualization of biological processes helps elucidate the metabolic state of breast cancer, while receptor-targeted radionuclide molecular imaging is sensitive and specific for visualization of the overexpressed molecular markers in breast cancer, contributing to early diagnosis and better management of cancer patients. The rapid development of radionuclide probes aids the diagnosis of breast cancer in various aspects. These probes target metabolism, amino acid transporters, cell proliferation, hypoxia, estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2), gastrin-releasing peptide receptor (GRPR) and so on. This article provides an overview of the development of radionuclide molecular imaging techniques present in preclinical or clinical studies, which are used as tools for early breast cancer diagnosis.

scholarly journals The incidence, prevalence, and survival of systemic sclerosis in the UK Clinical Practice Research Datalink

2018 ◽  
Vol 37 (8) ◽  
pp. 2103-2111 ◽  
Author(s):  
Jeremy G. Royle ◽  
Peter C. Lanyon ◽  
Matthew J. Grainge ◽  
Abhishek Abhishek ◽  
Fiona A. Pearce
Keyword(s):  
Clinical Practice ◽  
Systemic Sclerosis ◽  
Practice Research ◽  
Clinical Practice Research Datalink ◽  
The Uk ◽  
Incidence Prevalence

The UK COVID-19 contact tracing app as both an emerging technology and public health intervention: The need to consider promissory discourses

2021 ◽  
pp. 136345932110607
Author(s):  
Gabrielle Samuel ◽  
Rosie Sims
Keyword(s):  
Public Health ◽  
Rapid Development ◽  
Public Health Intervention ◽  
Health Intervention ◽  
Contact Tracing ◽  
The Public ◽  
Sociology Of Expectations ◽  
Short Period ◽  
The Media ◽  
The Uk

The UK’s National Health Service (NHS) COVID-19 contact tracing app was announced to the British public on 12th April 2020. The UK government endorsed the app as a public health intervention that would improve public health, protect the NHS and ‘save lives’. On 5th May 2020 the technology was released for trial on the Isle of Wight. However, the trial was halted in June 2020, reportedly due to technological issues. The app was later remodelled and launched to the public in September 2020. The rapid development, trial and discontinuation of the app over a short period of a few months meant that the mobilisation and effect of the discourses associated with the app could be traced relatively easily. In this paper we aimed to explore how these discourses were constructed in the media, and their effect on actors – in particular, those who developed and those who trialled the app. Promissory discourses were prevalent, the trajectory of which aligned with theories developed in the sociology of expectations. We describe this trajectory, and then interpret its implications in terms of infectious disease public health practices and responsibilities.

Editorial

2004 ◽  
Vol 3 (2) ◽  
Author(s):  
Chris Roseveare ◽  
Keyword(s):  
Bacterial Meningitis ◽  
Medical Staff ◽  
Case Reports ◽  
Rapid Development ◽  
Working Party ◽  
Significant Shift ◽  
Multiple Choice Questions ◽  
Acute Medicine ◽  
Junior Staff ◽  
The Uk

Over the past three years it has become apparent to me that referring to ‘current affairs’ in these columns can be a mistake, serving only to highlight inevitable printing delays. By the time this edition arrives on your doormat Euro 2004, ‘Big Brother’ and the early summer heat wave will be nothing but a distant memory. However the ‘recent’ publication of the Royal College of Physicians document ‘Acute Medicine – making it work for patients’ cannot be allowed to pass without a mention. This report represents a significant shift in the position of the College in relation to Acute Medicine since the previous working party reported its findings in 2000. The value of consultants specialising in Acute Medicine is now clearly recognised and supported – every trust should now have one, with the minimum figure of three per hospital being proposed by 2008. Whether this is achievable will depend on the rapid development of training schemes across the UK, as well as the generation of enthusiasm for the specialty amongst junior staff. The number of applicants for our Wessex programme indicates no shortage of the latter. Although developing a training scheme takes a lot of hard work, it is vital that those already working in the specialty make this a high priority. We have already seen benefits from the appointment of high quality middle grade staff and are looking forward to a ‘flood’ of future applicants for local consultant posts, 4 years from now. This edition comprises four more important review papers on aspects of acute medicine, along with the first in our ‘Controversies in acute medicine’ series. The latter was designed to try to stir up some correspondence, for future publication. The confusion over oxygen delivery in the acute setting seems to reign fairly widely amongst junior, and indeed some more senior medical staff. Hopefully Dr Cooper’s well-written paper will serve to dismiss some of the misconceptions in this area. Our reviews cover relatively uncommon, but nonetheless important aspects of acute medicine. Tuberculosis and HIV are both on the increase in the UK. The success of anti-retroviral therapy will undoubtedly lead HIVrelated illness to be a significant part of our practice over the next decade. An understanding of the range of conditions specific to this group of immunocompromised patients is therefore crucial for physicians involved in the acute take. Hypoglycaemia and suspected bacterial meningitis are both conditions which require immediate action by medical staff. Both of these reviews comprehensively cover their respective topics with a combination of well written text, illustrations tables and algorithms. Dr Hartman highlights recent evidence supporting the use of dexamethosone in bacterial meningitis and re-iterates some of the points made in an earlier edition regarding the use (and abuse) of CT scanning prior to lumbar puncture. For a change we have no case reports this time, although Dr Macdonald’s audit of the innovative review clinic in the Emergency Assessment Area of Heartlands hospital provides a worthy substitute. Submission of similar articles in future would be most welcome. Once again, a reminder that multiple choice questions are for self assessment and ‘personal’ CPD only; I hope you will find this edition helpful in your clinical practice.

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