Systemic sclerosis with hemoptysis and a huge lung cavity

Systemic sclerosis or scleroderma is associated with distal vasculitis, Raynaud’s phenomenon, and inflammation of internal organs and the skin. We present on a 58-year-old Thai woman with systemic sclerosis who came to the 10th Zonal Tuberculosis and Chest Disease Center, Chiang Mai, Thailand in 2009 and presented with hemoptysis and a solitary hugelung cavity as the predominant clinical manifestations which spontaneously resoluted 2 months later. This case demonstrates a solitary huge-lung cavity with hemoptysis and looked like from non-tuberculous Mycobacterial infections or malignancy with spontaneous resolution of hemoptysis and the lung cavity, which does not need invasive investigations.

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Raynaud's phenomenon

British Journal of Hospital Medicine ◽

10.12968/hmed.2019.80.11.658 ◽

2019 ◽

Vol 80 (11) ◽

pp. 658-664

Author(s):

Vikrant Devgire ◽

Michael Hughes

Keyword(s):

Systemic Sclerosis ◽

Surgical Intervention ◽

Raynaud’S Phenomenon ◽

Clinical Manifestations ◽

Raynaud's Phenomenon ◽

First Line ◽

Wide Range ◽

Significant Burden ◽

Associated Conditions ◽

First Line Treatment

Raynaud's phenomenon is a common vasospastic condition which carries a significant burden of pain and hand-related disability ( Hughes and Herrick, 2016 ). The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% ( Garner et al, 2015 ). Raynaud's phenomenon can occur either as a primary (‘idiopathic’) phenomenon or secondary to a wide range of underlying medical conditions and drug causes. Therefore, hospital-based specialists are frequently involved in the care of patients with Raynaud's phenomenon and need to be aware of associated conditions and prescribed medications for Raynaud's phenomenon. In particular, Raynaud's phenomenon is often the earliest manifestation of an underlying autoimmune connective tissue disease (e.g. systemic sclerosis). A comprehensive clinical assessment is required including performing targeted investigations (e.g. nailfold capillaroscopy and systemic sclerosis-associated autoantibodies). Patient education and lifestyle adaptations is first-line treatment for Raynaud's phenomenon. There is a wide range of pharmacological options including oral and intravenous drug therapies available to treat Raynaud's phenomenon. Surgical intervention is sometimes required for refractory Raynaud's phenomenon and tissue ischaemia. This review describes the clinical manifestations of Raynaud's phenomenon including potential secondary causes and presents an approach to assessment and management.

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Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis

International Journal of Rheumatology ◽

10.1155/2011/201787 ◽

2011 ◽

Vol 2011 ◽

pp. 1-7 ◽

Cited By ~ 13

Author(s):

Kait Arefiev ◽

David F. Fiorentino ◽

Lorinda Chung

Keyword(s):

Systemic Sclerosis ◽

Case Reports ◽

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Digital Ulcers ◽

Endothelin Receptor Antagonists ◽

Endothelin Receptor ◽

Significant Morbidity ◽

Receptor Antagonists ◽

Internal Organs

Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.

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SAT0318 GENERALIZED INDICATOR OF RAYNAUD’S PHENOMENON EXPRESSION FOR EVALUATION OF CLINICAL EFFICACY OF PROSTANOID THERAPY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2263 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1104.1-1105

Author(s):

I. Gaisin ◽

Z. Bagautdinova ◽

M. Glavatskikh ◽

N. Maximov ◽

R. Valeeva ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pain Intensity ◽

Raynaud’S Phenomenon ◽

Significant Proportion ◽

Clinical Manifestations ◽

Raynaud's Phenomenon ◽

Control Group ◽

Digital Ulcers ◽

High Level ◽

Generalized Indicator

Background:Raynaud’s phenomenon (RP) secondary to rheumatic diseases (RD) can progress to irreversible tissue damage with digital ulceration, scarring and, rarely, gangrene requiring amputation1. Current medical treatments for RP are far from ideal: they are often either ineffective and/or poorly tolerated, thus a significant proportion of patients discontinue drug therapy2.Objectives:To determine RP expression levels and to evaluate the long-term efficacy of iloprost and alprostadil in RP patients with RD.Methods:Indicated therapy with intravenous iloprost (n=10), alprostadil (n=17) or their combinations (n=13) was carried out for three years in patients with secondary RP in RD. Frequency of Raynaud’s attacks, digital ulcers (DU) formation and pain intensity on visual analogue scale (VAS) were evaluated. A control group included 30 patients with RP in RD who did not receive prostanoid therapy. By factor analysis method a generalized index of RP expression was identified, on the basis of which levels of RP expression were determined.Results:“RP expression” scale, revealed as an indicator of RP generalized manifestation, was an average value of two subscales: (1) consisted of 4 indices “DU”, “digital pitting scars”, “phalange amputation” and “frequency of Raynaud’s attack”, (2) included 3 indicators: “intensity of pain”, “duration of illness”, “whitening of fingers”. Correlation of subscales showed their reliability (r=0.294, p=0.053). RP final expression (severity) was 1.51±0.86. A low level of RP expression had values below 0.65, a high level – over 2.37. At baseline, the high level of RP severity was defined in 16 (22.9%) patients, medium – in 43 (61.4%), low – in 11 (15.7%).RP treatment with iloprost was effective in the healing of DU in 100% of patients and led to decrease of RP expression generalized index from 2.25 [1; 3] to 1.75 [1; 2] (p=0.012). Alprostadil therapy reduced pain intensity on VAS (p<0.05) and numbness during Raynaud’s attacks (p<0.01) and decreased RP expression from 1 [1; 2] to 1 [0.5; 1.5] (p=0.038). Patients on prostanoids combination had new DU and amputations; pain intensity reduced by 47% (p<0.05), RP expression generalized indicator did not change.Conclusion:Based on RP clinical manifestations in RD patients, a generalized index of RP expression was identified and levels of RP severity were determined. Treatment with iloprost or alprostadil has significant effects on reducing the clinical manifestations of RP with a corresponding decrease in its severity. Iloprost is indicated in patients with medium and high levels of RP expression index, alprostadil – with medium and low index and non-effectiveness of calcium channel blockers.References:[1]Hughes M, Herrick AL. Digital ulcers in systemic sclerosis.Rheumatology (Oxford) 2017;56 (1):14–25.[2]Kowal-Bielecka O, Fransen J, Avouac J et al. Update of EULAR recommendation for the treatment of systemic sclerosis.Ann Rheum Dis2017;76(8):1327–39.Acknowledgments:Professor LP. Anan’evaDisclosure of Interests:Ilshat Gaisin Speakers bureau: Boehringer Ingelheim, KRKA, Berlin-Chemie Menarini, Sanofi, Zukhra Bagautdinova: None declared, Marianna Glavatskikh: None declared, Nikolay Maximov Speakers bureau: Pfizer, KRKA, Rosa Valeeva: None declared, Oxana Desinova: None declared, Rushana Shayakhmetova: None declared

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

International Journal for Vitamin and Nutrition Research ◽

10.1024/0300-9831.73.1.3 ◽

2003 ◽

Vol 73 (1) ◽

pp. 3-7 ◽

Cited By ~ 22

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.

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