scholarly journals Nail-fold capillaroscopy for the dermatologists

2021 ◽  
Vol 0 ◽  
pp. 1-14
Author(s):  
Chander Grover ◽  
Deepak Jakhar ◽  
Arzoo Mishra ◽  
Archana Singal
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Diagnostic Utility ◽  
The Past ◽  
Diagnosis And Prognosis ◽  
Current Review ◽  
Final Data ◽  
Pubmed Search

Nail fold is one of the most accessible sites for studying changes in the microcirculation in various microangiopathies. The characterization of changes in microvasculature can provide useful clues towards the diagnosis and prognosis of a disease. The diagnostic utility of nail fold capillaroscopy has improved and expanded over the past couple of decades. Beyond connective tissue diseases, it is now explored for its role in various systemic and dermatological diseases. Incorporation of nail-fold capillaroscopy in the diagnostic criteria of systemic sclerosis has generated interest among dermatologists. The current review is aimed at providing knowledge about nail-fold capillaroscopy to dermatologists. For the purpose of review, a PubMed search was done using the keywords “nail fold capillaries” and “nail fold capillaroscopy”. All the articles were retrieved and classified into reviews and clinical studies of various types. The final data were then analyzed and presented in a narrative fashion.

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

Genetics of connective tissue diseases

2013 ◽  
Author(s):  
Myles Lewis ◽  
Tim Vyse
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Large Scale ◽  
Apoptotic Cell ◽  
Association Studies ◽  
Group Analysis ◽  
Connective Tissue Diseases ◽  
Susceptibility Genes ◽  
Type I ◽  
Genome Wide Association Studies

The advent of genome-wide association studies (GWAS) has been an exciting breakthrough in our understanding of the genetic aetiology of autoimmune diseases. Substantial overlap has been found in susceptibility genes across multiple diseases, from connective tissue diseases and rheumatoid arthritis (RA) to inflammatory bowel disease, coeliac disease, and psoriasis. Major technological advances now permit genotyping of millions of single nucleotide polymorphisms (SNPs). Group analysis of SNPs by haplotypes, aided by completion of the Hapmap project, has improved our ability to pinpoint causal genetic variants. International collaboration to pool large-scale cohorts of patients has enabled GWAS in systemic lupus erythematosus (SLE), systemic sclerosis and Behçet's disease, with studies in progress for ANCA-associated vasculitis. These 'hypothesis-free' studies have revealed many novel disease-associated genes. In both SLE and systemic sclerosis, identified genes map to known pathways including antigen presentation (MHC, TNFSF4), autoreactivity of B and T lymphocytes (BLK, BANK1), type I interferon production (STAT4, IRF5) and the NFκ‎B pathway (TNIP1). In SLE alone, additional genes appear to be involved in dysregulated apoptotic cell clearance (ITGAM, TREX1, C1q, C4) and recognition of immune complexes (FCGR2A, FCGR3B). Future developments include whole-genome sequencing to identify rare variants, and efforts to understand functional consequences of susceptibility genes. Putative environmental triggers for connective tissue diseases include infectious agents, especially Epstein-Barr virus; cigarette smoking; occupational exposure to toxins including silica; and low vitamin D, due to its immunomodulatory effects. Despite numerous studies looking at toxin exposure and connective tissue diseases, conclusive evidence is lacking, due to either rarity of exposure or rarity of disease.

scholarly journals Antifibrillarin autoantibodies present in systemic sclerosis and other connective tissue diseases interact with similar epitopes.

1995 ◽  
Vol 181 (3) ◽  
pp. 1027-1036 ◽  
Author(s):  
K N Kasturi ◽  
A Hatakeyama ◽  
H Spiera ◽  
C A Bona
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Competitive Inhibition ◽  
Molecular Mimicry ◽  
Connective Tissue Diseases ◽  
Nuclear Antigen ◽  
Barr Virus ◽  
Recombinant Fusion Proteins ◽  
Epstein Barr

Autoantibodies specific against fibrillarin, a 34-kD nucleolar protein associated with U3-snRNP, are present in patients with systemic sclerosis (SSc). To understand the mechanisms involved in the induction of these autoantibodies, we prepared a series of human fibrillarin recombinant proteins covering the entire molecule and analyzed their interaction with the autoantibodies present in various connective tissue diseases. Our results showed that antifibrillarin autoantibodies are present not only in SSc, as previously reported, but also in a variety of other connective tissue diseases. Patients with SSc (58%), mixed connective tissue diseases (60%), CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dismotility, sclerodactyly, and telangiectasia syndrome) (58%), systemic lupus erythematosus (39%), rheumatoid arthritis (60%), and Sjogern's syndrome (84%) showed presence of antifibrillarin autoantibodies. Results obtained from competitive inhibition radioimmunoassay and Western blot analyses with purified recombinant fusion proteins revealed that these autoantibodies react primarily with epitope(s) present in the NH2- (AA 1-80) and COOH-terminal (AA 276-321) domains of fibrillarin. Autoantibodies reacting with internal regions of fibrillarin are less frequent. Analysis of the hydrophilicity profiles of reactive peptides showed presence of three potential antigenic sites in the NH2- and two in the COOH-terminal regions. While a hexapeptide sequence NH2 terminus of fibrillarin is shared with an Epstein-Barr virus-encoded nuclear antigen, the COOH-terminal region shares sequence homology with P40, the capsid protein encoded by herpes virus type 1. Interestingly, these two regions of fibrillarin also contain the most immunodominant sequences, as predicted by surface probability and the Jameson and Wolf antigenic index. These observations suggest that molecular mimicry might play an important role in the induction of antifibrillarin autoantibodies.

scholarly journals Stanley Miles Partridge, 2 August 1913 - 26 April 1992

1994 ◽  
Vol 40 ◽  
pp. 327-346
Keyword(s):  
Connective Tissue ◽  
Chondroitin Sulphate ◽  
Basic Structure ◽  
Side Chains ◽  
Unusual Structure ◽  
Protein Core ◽  
The Past ◽  
Initial Representation ◽  
Bottle Brush

The explosion of interest in the connective-tissue field over the past decade is based on the early intellectual contributions of just a few pioneering investigators. Miles Partridge was one of those few. He will be best remembered for the characterization of elastin from a histological entity to a protein in its own right, and for demonstrating how its unusual structure results in a rubber-like elasticity. He also identified the glycoprotein in aortic elastin that was later shown to exist as microfibrils and to be involved in directing the elastin fibre during development. He made a major contribution to the elucidation of the basic structure of the glycosaminoglycans by his initial representation of chondroitin sulphate as a bottle-brush structure consisting of a protein core and numerous side chains of polysaccharides. He chose these topics firstly because very little was known about these structures and they were considered by his contemporaries to be insoluble problems, and secondly because it was not in his nature just to extend or confirm other people’s work. Miles Partridge clearly possessed a vision of connective tissue well ahead of his time, and his legacy has been to provide openings into areas previously thought to be intractable. He can therefore rightly be considered one of the fathers of connective-tissue biochemistry.

Ulnar artery vasculopathy: a common but nonspecific feature of systemic sclerosis

2017 ◽  
Vol 2 (3) ◽  
pp. 221-224
Author(s):  
Tae-Jong Kim ◽  
Soon-Young Song ◽  
Hee Chang Ahn ◽  
Yoon-Kyoung Sung ◽  
Sang-Cheol Bae ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Case Series ◽  
Ulnar Artery ◽  
Common Finding ◽  
Clinical Factors ◽  
Vessel Disease ◽  
Large Vessel Disease

Introduction Systemic sclerosis (SSc) is characterized by microvascular abnormalities and fibrosis. Several studies have reported that large vessel disease is also common in SSc. The aim of this case series was to investigate whether ulnar artery involvement in patients with SSc is a disease-specific phenomenon, as compared to other connective tissue diseases (CTD). Methods A total of 28 patients, including 7 with SSc and 12 with systemic lupus erythematosus (SLE), underwent brachial arteriography due to severe Raynaud's phenomenon and/or digital ulcerations and were enrolled in the study. They were divided into two groups: an SSc/SSc-overlap group and a non-SSc group. The collection of the clinical parameters was conducted to investigate the associations between clinical factors and the ulnar artery vasculopathy. Results The SSc/SSc-overlap group (n = 10) consisted of 7 patients with SSc and 3 with features overlapping SSc. In the non-SSc group (n = 18), 12 cases of SLE, 2 of mixed connective tissue disease, 1 of dermatomyositis + SLE, 1 of rheumatoid arthritis, 1 of Sjogren's syndrome, and 1 case of skin vasculitis, were included. The relative frequencies of ulnar artery involvement were not significantly different between the SSc/SSc-overlap and non-SSc groups, respectively (n = 6, 60% vs. n = 9, 50%, p = 0.611). Conclusions Although ulnar artery involvement was frequently detected in patients with SSc/SSc-overlap, it was also a common finding in other CTDs; therefore, it is not specific to SSc or SSc-overlap diseases.

Quantification and molecular characterization of regulatory T cells in connective tissue diseases

Autoimmunity ◽  
2009 ◽  
Vol 42 (1) ◽  
pp. 41-49 ◽  
Author(s):  
Leontina Banica ◽  
Leontina Banica ◽  
Alina Besliu ◽  
Leontina Banica ◽  
Alina Besliu ◽  
...  
Keyword(s):  
T Cells ◽  
Connective Tissue ◽  
Regulatory T Cells ◽  
Molecular Characterization ◽  
Connective Tissue Diseases

scholarly journals Nailfold Capillaroscopy in Rheumatic Diseases

2020 ◽  
Author(s):  
Abhishek Patil ◽  
Isha Sood
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Early Diagnosis ◽  
Rheumatic Diseases ◽  
Connective Tissue Diseases ◽  
Nailfold Capillaroscopy ◽  
Indispensable Tool

Nailfold capillaroscopy (NFC) has developed into an indispensable tool for rheumatologists in the evaluation of rheumatic diseases. It offers various advantages in being rapid, noninvasive, and inexpensive. With NFC we are able to visualize the microcirculatory changes in the nail beds. These changes are key to the pathogenesis of connective tissue diseases such as systemic sclerosis. Hence NFC helps in early diagnosis of various connective tissue diseases. There is a lack of standardization in the techniques used and various capillary parameters studied, which could lead to variation in the reporting of the parameters studied. In this chapter we shall try to highlight the most common parameters studied in capillaroscopy and its utility in various connective tissue diseases.

scholarly journals A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

2020 ◽  
Vol 6 (4) ◽  
pp. 532
Author(s):  
Neha T. Solanki ◽  
Sahana P. Raju ◽  
Deepmala Budhrani ◽  
Bharti K. Patel
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Tertiary Care ◽  
Connective Tissue Diseases ◽  
Cutaneous Manifestations ◽  
Respiratory Involvement

<p class="abstract"><strong>Background:</strong> The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.</p><p class="abstract"><strong>Methods:</strong> The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.<strong></strong></p><p class="abstract"><strong>Results:</strong> The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc)  and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.</p><p class="abstract"><strong>Conclusions:</strong> AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.</p>

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