scholarly journals Systemic Sclerosis in Zimbabwe: Autoantibody Biomarkers, Clinical, and Laboratory Correlates

2021 ◽  
Vol 12 ◽  
Author(s):  
Elopy N. Sibanda ◽  
Yvonne Dube ◽  
Mazvita Chakawa ◽  
Takafira Mduluza ◽  
Francisca Mutapi

IntroductionSystemic sclerosis (SScl) is an autoimmune disease whose prevalence is rarely reported in Africa. Autoantibodies are the biomarkers of the condition, precede overt disease and determine disease phenotypes. SSc specific autoantibodies also vary between racial groupings. Objective: To investigate the clinical and laboratory characteristics of Zimbabwean patients who were reactive SSc specific autoantibodies.Materials and Method240 patients, 173 of them female with SSc specific autoantibodies were included. Autoantibodies were detected by indirect immunofluorescence microscopy and immunoblotting using a panel of 13 SScl (Euroimmun Ag., Germany). Demographic, clinical and laboratory parameters relevant to the monitoring of SScl were captured. These included pulmonary function tests, hematology, clinical chemistry, serology and thyroid function tests. Allergy skin prick tests (SPT) to inhalant and food allergen sources were conducted when indicated.ResultsAll the 240 patients (median age was 36 years) expressed SSc specific autoantibodies. 86% were Black, 11% White and 3% Asian and a fifth (20%) were younger than 16 years. Eleven (4.6%) fulfilled the ACR/EULAR classification of SSc. Clinically they had limited cutaneous (n=6), diffuse cutaneous (n=3) and SScl/inflammatory myopathy overlap (n=2). The most frequently detected antibodies anti-RNA polymerase III (RNAP) 55%, anti-Th/To (28%) anti-RNAP 11 (22%), anti-CENPB (18%) and anti-Scl-70/ATA (13%). Racial variations in the expression of these antibodies were apparent between Black, White and Asian patients. The majority (95%), who did not fulfil the ARA/EULAR criteria were symptomatic. Raynaud’s Phenomenon was documented in 24%. Respiratory symptoms included coughing, dyspnea and wheezing. There was a restrictive ventilatory defect with increased FEV1/FVC ratio. Pruritus, urticaria and skin depigmentation were the main cutaneous features while constipation, bloating, Gastroesophageal reflux disease (GERD) and abdominal pain dominated GI symptoms. Mean blood pressure readings while normal varied with biomarkers. Haematology and biochemistry parameters were within normal reference ranges.ConclusionThe expression of SSc specific autoantibodies is common and associated with known SSc symptoms. The types and frequency of autoantibodies varied with racial groupings. A fifth of the patients were children below the age of 16 years.

2006 ◽  
Vol 63 (4) ◽  
pp. 163-165 ◽  
Author(s):  
M. Alqahatani ◽  
W. Tamimi ◽  
M Aldaker ◽  
F. Alenzi ◽  
H. Tamim ◽  
...  

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Francesco Masini ◽  
Lucio Monaco ◽  
Klodian Gjeloshi ◽  
Emanuele Pinotti ◽  
Roberta Ferrara ◽  
...  

Abstract Background Systemic sclerosis (SSc) is a multi-system connective tissue disease characterized by microvascular damage, inflammation and fibrosis. The aim of our study is to investigate a possible correlation between nailfold videocapillaroscopy patterns and internal organ involvement in patients with early SSc. Methods We enrolled 40 patients with early SSc according to LeRoy criteria and performed echocardiogram, pulmonary function tests (PFTs) and nailfold videocapillaroscopy. Results 20 patients had early pattern and 20 active pattern. A comparative analysis between SSc patients and healthy controls showed an alteration of mitral annular plane systolic excursion (MAPSE) (1.5±0.1 vs 1.7±0.1; p < 0.001), respectively; an increased occurrence of diastolic dysfunction (11/40 vs 0/21; p = 0.01) respectively; a higher values of systolic pulmonary arterial pression (PAPs) (32±6 vs 22±6; p < 0.001) and a higher frequency of patients with pulmonary hypertension (12/40 vs 0/21; p = 0.005) in SSc group. The patients with an active capillaroscopic pattern showed a trend of reduction in MAPSE, diastolic dysfunction, contractility of the right ventricle and pulmonary function tests parameters. We observed a correlation between myocardial and pulmonary vascular damage and between myocardial and pulmonary fibrosis, with lower DLCO value in patients with diastolic dysfunction (64±17 vs 85±9; p < 0.0001). Conclusion A subclinical myocardial damage in these patients may be observed also in the absence of symptoms. In fact, in our study it can be noted that asymptomatic patients present a reduction in the linear myocardial contractility. Furthermore, we observed the presence of grade I diastolic dysfunction in 27.5% of cases, a sign of an initial stiffening of the wall, likely due to a fibrotic damage. In our study linear contractility emerges to be not correlated with the presence of diastolic dysfunction. Such data is very intriguing if we consider that in many studies the resolution of linear contractility with conserved ejection fraction correlated with subendocardial damage induced by microcirculation alterations. The hypothesis is the presence of a double framework at the myocardial level: a vascular damage and the presence of increased inflammation and fibrosis. The presence fibrosis is correlated between the various organs, with the values of DLCO strongly correlating with the presence of diastolic dysfunction; instead DLCO values do not correlate with pulmonary pressure values. The analysis of the capillaroscopic parameters we observed how in the early pattern the presence of vascular damage with MAPSE and PAPs alterations and presence of pulmonary hypertension emerge, while passing from early to active the presence of fibrotic damage becomes evident, the diastolic dysfunction passing from 15% to 40% and DLCO passing from 83% to 75%. The presence of subclinical cardiac and pulmonary damage in patients with systemic sclerosis and that this damage is closely related to the capillaroscopic pattern. Disclosures F. Masini: None. L. Monaco: None. K. Gjeloshi: None. E. Pinotti: None. R. Ferrara: None. T. Salvatore: None. G. Cuomo: None.


2018 ◽  
Vol 15 (3) ◽  
pp. 117
Author(s):  
Vijaya Sarathi ◽  
KSandhya Rani ◽  
Sunanda Tirupati ◽  
KDileep Kumar

2009 ◽  
Vol 44 (12) ◽  
pp. 1226-1234 ◽  
Author(s):  
Serena Panigada ◽  
Angelo Ravelli ◽  
Michela Silvestri ◽  
Claudio Granata ◽  
Silvia Magni-Manzoni ◽  
...  

2018 ◽  
Vol 27 (148) ◽  
pp. 170102 ◽  
Author(s):  
Melissa Caron ◽  
Sabrina Hoa ◽  
Marie Hudson ◽  
Kevin Schwartzman ◽  
Russell Steele

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO. Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.


1991 ◽  
Vol 71 (2) ◽  
pp. 438-444 ◽  
Author(s):  
H. M. Hollingsworth ◽  
M. R. Pratter ◽  
J. M. Dubois ◽  
L. E. Braverman ◽  
R. S. Irwin

To determine whether thyrotoxicosis has an effect on the asthmatic state in subjects with mild asthma, airway responsiveness, lung function, and exercise capacity were measured in a randomized double-blind placebo-controlled trial before and after liothyronine (triiodothyronine, T3)-induced thyrotoxicosis. Baseline evaluation of 15 subjects with mild asthma included clinical evaluation, thyroid and routine pulmonary function tests, airway responsiveness assessment by methacholine inhalation challenge, and a symptom-limited maximal exercise test. For all subjects, the initial testing revealed that the dose of methacholine which provoked a 20% fall in forced expiratory volume in 1s (PD20) was in a range consistent with symptomatic asthma. There was no significant change in pulmonary function tests, airway reactivity (PD20), or exercise capacity in either the placebo or the T3-treated groups. Thyroid function tests confirmed mild sustained thyrotoxicosis in the T3-treated groups. We conclude that mild T3-induced thyrotoxicosis of 4-wk duration had no effect on lung function, airway responsiveness, or exercise capacity in subjects with mild asthma.


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