scholarly journals P032 Increased incidence of anti-synthetase syndrome during COVID-19 pandemic

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Bethan Phillips ◽  
James Martin
Keyword(s):  
Clinical Features ◽  
Raynaud’S Phenomenon ◽  
Lower Lobe ◽  
Raynaud's Phenomenon ◽  
Muscle Disease ◽  
Clinical Impression ◽  
Health Board ◽  
Shortness Of Breath ◽  
Number Of Patients ◽  
Mechanic’S Hands

Abstract Background/Aims  Anti-synthetase syndrome is an idiopathic inflammatory muscle disease, characterised by anti-Jo1 antibodies. Clinical presentation varies between individuals. The diagnosis requires the detection of one or more anti-synthetase antibodies and one or more of the following clinical features: interstitial lung disease, myositis, and inflammatory polyarthritis. Other supporting features include fever, Raynaud’s phenomenon, and 'mechanic’s hands'. The severity and extent of pulmonary involvement typically determines prognosis. It is a rare disease, with estimated prevalence of 1.5 cases per 100,000. Our aim is to report an increase in the local prevalence, which coincides with the COVID-19 pandemic. Methods  We retrospectively reviewed all new diagnoses of anti-synthetase syndrome made between March and July 2020 at the Royal Glamorgan Hospital. Results  3 new diagnoses of anti-synthetase syndrome were made during the study period. Based on the reported prevalence, we expect to see around 2 cases every 6-months in our hospital (which serves a population of around 300,000.) Anecdotally, the incidence has been much lower than this previously, and in the experience of three rheumatology consultants working at this hospital, only 6 diagnoses have been made over the last 15 years. This represents a 15-fold increase in the annual diagnosis rate. This anecdotal rise in the number of patients with anti-synthetase syndrome coincides with the COVID-19 pandemic. The incidence of COVID-19 in our health-board during this time period was 666 per 100,000. This is higher than the national incidence (486 per 100,000.) All three patients diagnosed with anti-synthetase syndrome had prominent symptoms of shortness of breath, hypoxia and presented on multiple occasions before the final diagnosis was made. Notably, all patients presented acutely unwell with severe symptoms, where ordinarily this diagnosis would be made in the outpatient setting. One case presented with a typical constellation of signs and symptoms. Specifically, progressive shortness of breath with evidence of bilateral interstitial pulmonary disease on imaging, myositis and anti-Jo1 positivity. The remaining two cases presented with primarily lung involvement. In both cases, chest imaging demonstrated extensive bilateral lower lobe pulmonary infiltrates, reported as being in-keeping with COVID-19. Despite repeated negative COVID-19 PCR results, the clinical impression was of probable COVID-19 infection. Following repeated presentations with worsening respiratory symptoms, specialist review was arranged, and ultimately anti-synthetase syndrome was diagnosed. With hindsight, one patient demonstrated extrapulmonary features of Raynaud’s phenomenon and 'mechanic’s hands'. All cases received methylprednisolone and mycophenolate. Conclusion  We report an anecdotal local increase in the incidence of anti-synthetase syndrome, coinciding with the COVID-19 pandemic. The cases described demonstrate an overlap between the presenting clinical features of COVID-19 and anti-synthetase syndrome, and that due to high clinical suspicion of COVID-19 in the context of a global pandemic, some diagnoses of anti-synthetase syndrome may be being missed or delayed. Disclosure  B. Phillips: None. J. Martin: None. C. Rhys-Dillon: None.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078
Author(s):  
Ariane Herrick ◽  
Michael Hughes
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for example vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

scholarly journals FRI0227 A USABILITY SURVEY OF WRIST MOUNTED DISPOSABLE HEAT PAD ON RAYNAUD’S PHENOMENON IN PATIENTS WITH CONNECTIVE TISSUE DISEASES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 696.1-697
Author(s):  
N. Azuma ◽  
T. Furukawa ◽  
Y. Shima ◽  
K. Matsui
Keyword(s):  
Connective Tissue ◽  
Daily Life ◽  
Wrist Joint ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Research Support ◽  
Heating Method ◽  
Number Of Patients ◽  
Associated Symptoms

Background:For patients with connective tissue diseases (CTD), vasodilators are used to treat Raynaud’s phenomenon (RP), they are difficult to control only by medication. Although physicians recommend the use of a portable handwarmer or gloves to patients with CTD presenting with RP, sustained heat-retention effects cannot be obtained from them because the patients’ daily life-related activities prevent their continued use. Since the wrist mounted disposable heat pad maintains the degrees of freedom of the hands and fingers and can remain usable during the daily activities, we considered this heat pad as a useful and highly practical heating method for CTD patients presenting with RP.Objectives:To investigate the usability and changes in symptoms resulting from the use of the wrist mounted disposable heat pad in CTD patients presenting with RP.Methods:Subjects were 23 outpatients with CTD presenting with RP (23 females; mean age 62.6 years; mean duration following the onset of RP 10.3 years; 12 systemic sclerosis, 5 mixed connective tissue disease, 5 Sjögren’s syndrome, and 1 systemic lupus erythematosus) who had used the wrist mounted disposable heat pad (put the pad in a specifically designed holder and wrap it around wrist joint (max. temperature 42 degrees Celsius, heat-retention time 6 hours)). We investigated through interviews with them the use situations, usability, and changes in RP. During their using the heat pad, medication and daily life-related precautions against RP continued to be implemented as before.Results:Many patients had no knowledge of the heat pad (n=17, 73.9%). The most common wearing time of the heat pad was 5–6 hours (n=8, 34.8%). As for scenes of wearing the heat pad, patients who wore the pad when being out of the home accounted for the highest proportion (n=16, 69.6%), and as follows: at home (n=6, 26.1%), during kitchen work (n=3, 13.0%), and during housework (n=2, 8.7%). 17 patients (73.9%) replied that usability was “good”, and 18 (78.3%) replied that usability was “better” compared with conventional measures. Moreover, many patients (n=16, 69.6%) replied that RP and associated symptoms had become reduced or alleviated. No patients replied that RP and associated symptoms had become exacerbated or severer. In terms of advantages of using the heat pad, patients who replied that the site on which the pad was mounted was felt to be warm accounted for the highest proportion (n=8, 34.8%), and those who replied that sites other than where the pad was mounted (such as fingertips, hands, and arms) were also warmed accounted for virtually the same proportion (n=7, 30.4%). Over 60% of the patients (n=14, 60.9%) replied that symptoms associated with RP (skin color, cold sensation, and pain) had become reduced or disappeared. In terms of disadvantages of using the heat pad, patients who replied that it was bothersome to use the pad accounted for the highest proportion while other patients made replies referring to cost and bad appearance. No significant accident occurred and as many as 17 patients (73.9%) replied that they would like to continue to use the heat pad in the future.Conclusion:There have been few reports evaluating the usefulness of a heat pad for RP. The wrist mounted disposable heat pad was thought to be a heating method having the potential to achieve high levels of usability and practicality on CTD patients presenting with RP. Given that the heat pad alleviated RP or caused sites other than where the pad was mounted to be felt warm even though it did not directly heat the hands and fingers, the pad seemed to have usefulness attributed to the heating of the wrist. Although the heat pad seems to be an excellent method for addressing RP in patients’ daily lives, we hope that this heat pad will be evaluated on a larger number of patients with the addition of objective indices.References:[1]Koscheyev VS, et al. Aviat Space Environ Med. 72: 713-719, 2001.Disclosure of Interests:Naoto Azuma: None declared, Tetsuya Furukawa: None declared, Yoshihito Shima Grant/research support from: Endowed chair funded by/accepted a researcher from Kirikai Chemical and Kobayashi Pharmaceutical., Kiyoshi Matsui Grant/research support from: Asahi Kasei Pharma, Astellas Pharma (research grants), Speakers bureau: Bristol-Myers Squibb (lecture fees)

scholarly journals The influence of Multiwave Locked System (MLS) laser therapy on clinical features, microcirculatory abnormalities and selected modulators of angiogenesis in patients with Raynaud’s phenomenon

2014 ◽  
Vol 34 (3) ◽  
pp. 489-496 ◽  
Author(s):  
Anna Kuryliszyn-Moskal ◽  
Jacek Kita ◽  
Agnieszka Dakowicz ◽  
Sylwia Chwieśko-Minarowska ◽  
Diana Moskal ◽  
...  
Keyword(s):  
Laser Therapy ◽  
Clinical Features ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon

Diagnostic algorithm for Raynaud’s phenomenon and vascular skin lesions in systemic lupus erythematosus

Lupus ◽  
2010 ◽  
Vol 19 (9) ◽  
pp. 1087-1095 ◽  
Author(s):  
JG Richter ◽  
O. Sander ◽  
M. Schneider ◽  
P. Klein-Weigel
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
Diagnostic Algorithm ◽  
Skin Lesions ◽  
Raynaud's Phenomenon ◽  
Systemic Lupus

Skin discolorations and skin lesions due to vascular pathologies are common clinical features in systemic lupus erythematosus. A variety of clinical manifestations such as Raynaud’s phenomenon, acrocyanosis, livedo patterns, erythematous or violaceous macules and papules or necrosis are triggered by heterogeneous pathophysiological mechanisms such as vasospasm, vasculitis or thromboembolism. A standardized macro- and microvascular assessment is necessary to establish the correct diagnosis. We describe and illustrate common clinical features of vascular skin manifestations in systemic lupus erythematosus and present a diagnostic algorithm. Lupus (2010) 19, 1087—1095.

scholarly journals The clinical manifestations at the onset of antisynthetase syndrome: A chameleon with multiple faces

Reumatismo ◽  
2020 ◽  
Vol 72 (2) ◽  
pp. 86-92
Author(s):  
A.C.C.D. Baccaro ◽  
G.L. Behrens Pinto ◽  
R.C.S. Carboni ◽  
S.K. Shinjo
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Symptoms ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Joint Involvement ◽  
Antisynthetase Syndrome ◽  
Initial Symptoms ◽  
Mechanic’S Hands

The antisynthetase syndrome (ASS) is clinically characterized by fever, myositis, interstitial lung disease, joint involvement, mechanic’s hands, or Raynaud’s phenomenon, and the presence of antisynthetase autoantibodies. These clinical manifestations may not occur simultaneously. Therefore, the aim of this study was to analyze the sequence in which these clinical manifestations can develop at the onset of ASS. This retrospective, single-center cohort study enrolled 55 ASS patients. Their mean age at the onset of ASS symptoms was 42.3±11.8 years. There was a predominance of female patients (75.9%) and white patients (72.7%). At initial presentation, 41.8% of the patients had fever, 43.6% had joint symptoms, 38.2% had myositis, 36.4% had interstitial lung disease, 18.2% had Raynaud’s phenomenon, and 16.4% had mechanic’s hands. Subsequent clinical symptoms emerged at varying time points. In two out of 55 cases, joint, muscle, and lung manifestations developed simultaneously. The median time between the onset of symptoms and the complete ASS clinical manifestation was 19.9 (4.0-60.2) months; whereas, the timeframe between the onset of symptoms and the ASS diagnosis was 29.0 (11.0-63.0) months. The confounding misdiagnoses interfering with the initial diagnosis were polymyositis (52.7%), dermatomyositis (29.1%), nonspecific interstitial pneumopathy (23.6%), rheumatoid arthritis (18.2%), and others (10.9%). Clinical features at the onset of ASS are highly variable. Consequently, confounding factors can lead to significant delays for the final and definitive diagnosis of ASS. Therefore, ASS should be considered a differential diagnosis in patients with initial symptoms of joint, lung, and/or muscle involvements, as well as fever, mechanic’s hands, and/or Raynaud’s phenomenon manifestations.

scholarly journals The prevalence and clinical features associated of hypothyroidism among Thai systemic sclerosis patients

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yathao Paolee ◽  
Chingching Foocharoen ◽  
Suranut Charoensri ◽  
Mayfong Mayxay ◽  
Ajanee Mahakkanukrauh ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Features ◽  
Subclinical Hypothyroidism ◽  
Raynaud’S Phenomenon ◽  
Sample Size Calculation ◽  
Raynaud's Phenomenon ◽  
Primary Hypothyroidism ◽  
Thyroid Function Tests ◽  
Historical Cohort ◽  
Duration Of Disease

AbstractThyroid disease, particularly hypothyroidism, has been reported in systemic sclerosis (SSc). Some clinical features of SSc can also present in hypothyroidism. Our aims were to determine the prevalence of, and describe clinical features associated with, hypothyroidism in SSc patients. We conducted a historical cohort study of adult SSc patients who underwent screening thyroid function tests at the Scleroderma Clinic, Khon Kaen University, Thailand, between 2009 and 2018. The patients who had any thyroid disorders before the onset of SSc and were diagnosed as an overlap syndrome were excluded. A total of 200 SSc were included according to sample size calculation, among whom the female to male ratio was 2:1. The majority of cases (137; 69.5%) were diffuse cutaneous SSc subset. The mean age was 55.8 ± 10.7 years and the median duration of disease 4.9 (IQR 1.6–9.9) years. Of the total, 9 had primary hypothyroidism (prevalence 4.5%; 95%CI 2.1–8.4) and 22 had subclinical hypothyroidism (prevalence 11%; 95%CI 7.0–16.2). Of the latter 22, 71% had dcSSc. Logistic regression analysis indicated that unexplained anemia was significantly associated with either subclinical hypothyroid or hypothyroidism (OR 2.74; 95% CI 1.17–6.47), whereas Raynaud’s phenomenon had a negative association (OR 0.28; 95% CI 0.11–0.66). Neither severity of skin tightness nor internal organ involvement were associated with hypothyroidism among SSc patients. Clinical-subclinical hypothyroidism is uncommon among SSc patients, it is frequently associated with anemia, and less so Raynaud’s phenomenon. Clinical-subclinical hypothyroidism should thus be considered in cases of unexplained anemia in SSc patients.

Ascorbic Acid does not Improve Endothelium-Dependent Flow-mediated Dilatation of the Brachial Artery in Patients with Raynaud’s Phenomenon Secondary to Systemic Sclerosis

2003 ◽  
Vol 73 (1) ◽  
pp. 3-7 ◽  
Author(s):  
M. E. Mavrikakis ◽  
J. P. Lekakis ◽  
M. Papamichael ◽  
K. S. Stamatelopoulos ◽  
Ch. C. Kostopoulos ◽  
...  
Keyword(s):  
Ascorbic Acid ◽  
Smooth Muscle ◽  
Systemic Sclerosis ◽  
Endothelial Dysfunction ◽  
Brachial Artery ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Water Soluble ◽  
Placebo Administration ◽  
Flow Mediated Dilatation

Previous studies have shown that patients with Raynaud’s phenomenon secondary to systemic sclerosis present abnormal endothelial function; the mechanisms responsible for the endothelial dysfunction are unknown but increased vascular oxidative stress could be a possible cause. The hypothesis that a potent water-soluble antioxidant can reverse endothelial dysfunction in these patients was tested in the present study. We examined 11 female patients with Raynaud’s phenomenon secondary to systemic sclerosis and ten healthy control women by ultrasound imaging of the brachial artery to assess flow-mediated (endothelium-dependent) and nitrate-induced (endothelium-independent) vasodilatation. Flow-mediated dilatation and nitrate-induced dilatation were significantly reduced in patients with Raynaud’s phenomenon, indicating abnormal endothelial and smooth muscle cell function. Patients with Raynaud’s phenomenon entered a double-blind, randomized, crossover placebo-controlled trial and received orally 2 g of ascorbic acid or placebo; vascular studies were repeated two hours after ascorbic acid or placebo administration. Flow-mediated dilatation did not improve after ascorbic acid (1.6 ± 2.2% to 2.2 ± 2.5%, ns) or placebo administration (1.2 ± 1,9% to 1.7 ± 1.4%, ns); also nitrate-induced dilatation was similar after ascorbic acid or placebo (16 ± 7.4% vs 17 ± 8%, ns), suggesting no effect of ascorbic acid on endothelial and vascular smooth muscle function. In conclusion, ascorbic acid does not reverse endothelial vasomotor dysfunction in the brachial circulation of patients with Raynaud’s phenomenon secondary to systemic sclerosis. The use of different antioxidants or different dosing of ascorbic acid may be required to show a beneficial effect on endothelial vasodilator function.

The response of skin perfusion and of rheological and immunological variables to intravenous prostanoid administration in Raynaud’s phenomenon secondary to collagenosis

VASA ◽  
2005 ◽  
Vol 34 (4) ◽  
pp. 243-249 ◽  
Author(s):  
Drinda ◽  
Neumann ◽  
Pöhlmann ◽  
Vogelsang ◽  
Stein ◽  
...  
Keyword(s):  
Laser Doppler Flowmetry ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Plasma Viscosity ◽  
Laser Doppler ◽  
Erythrocyte Aggregation ◽  
Control Group ◽  
Long Term Effects ◽  
Doppler Flowmetry

Background: Prostanoids are used in the treatment of Raynaud’s phenomenon and acral perfusion disorders secondary to collagenosis. In subjective terms, intravenous administration of these agents produces success in more than 50% of patients. The therapeutic outcome of clinical administration of alprostadil or iloprost may vary from individual to individual. Patients and methods: The following variables were analysed in a cross-over study in 27 patients with collagenosis and Raynaud’s phenomenon: plasma viscosity and erythrocyte aggregation (rheological variables), partial pressure of oxygen and laser Doppler flowmetry in the finger region, and lymphocyte phenotyping and interleukin (IL) determinations (immunological variables). Results: Laser Doppler flowmetry revealed significant differences between patients with secondary Raynaud’s phenomenon and a control group of 25 healthy subjects. Laser Doppler readings did not change significantly as a result of the treatments. Therapy with iloprost produced a reduction in IL-1beta, L-selectin (CD 62 L) and IL-6. Conclusion: The change in immunological variables due to iloprost may explain the long-term effects of prostaglandins in the treatment of Raynaud’s phenomenon. From our results it is not possible to infer any preference for iloprost or alprostadil.

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