Dyspnea In A Patient With Raynaud's Phenomenon: The Uncovering Of Interstitial Lung Disease (ILD)

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.

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GOOD LUNG FUNCTION BUT PROGRESSIVE RAYNAUD'S PHENOMENON IN A PATIENT OF SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE AFTER BILATERAL LUNG TRANSPLANTATION

Respirology ◽

10.1111/resp.13420_185 ◽

2018 ◽

Vol 23 ◽

pp. 157-157

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Function ◽

Lung Transplantation ◽

Lung Disease ◽

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Bilateral Lung Transplantation ◽

Bilateral Lung

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A Clinical Analysis of Risk Factors for Interstitial Lung Disease in Patients with Idiopathic Inflammatory Myopathy

Clinical and Developmental Immunology ◽

10.1155/2013/648570 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 9

Author(s):

Xiaomin Cen ◽

Chuan Zuo ◽

Min Yang ◽

Geng Yin ◽

Qibing Xie

Keyword(s):

Risk Factors ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Raynaud’S Phenomenon ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Raynaud's Phenomenon ◽

Clinical Analysis ◽

Laboratory Findings ◽

Rheumatology Unit

Interstitial lung disease (ILD) is a common and severe complication of idiopathic inflammatory myopathies (IIM). The aim of our study was to identify risk factors for ILD by evaluating both clinical and biochemical features in IIM patients with or without ILD. From January 2008 to December 2011, medical records of 134 IIM patients in our rheumatology unit were reviewed. The patients were divided into ILD group (83 patients) and non-ILD group (51 patients). The clinical features and laboratory findings were compared. The univariable analyses indicated that arthritis/arthralgia (54.2% versus 17.6%,P<0.05), Mechanic’s hand (16.9% versus 2.0%,P<0.05), Raynaud’s phenomenon (36.1% versus 2.0%,P<0.05), heliotrope rash (44.6% versus 19.6%,P<0.05), fever (43.4% versus 21.6%,P<0.05), elevated ESR (60.2% versus 35.3%,P<0.05), elevated CRP (55.4% versus 31.4%,P<0.05), or anti-Jo-1 antibody (20.5% versus 5.9%,P<0.05) were risk factors for developing ILD in IIM. Multivariable unconditional logistic regression analysis that showed arthritis/arthralgia (OR 7.1, 95% CI 2.8–18.1), Raynaud’s phenomenon (OR 29.1, 95% CI 3.6–233.7), and amyopathic dermatomyositis (ADM) (OR 20.2, 95% CI 2.4–171.2) were the independent risk factors for developing ILD in IIM.

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Intra-arterial administration of reserpine. Its use in patients with Raynaud's disease or Raynaud's phenomenon

Archives of Internal Medicine ◽

10.1001/archinte.125.5.825 ◽

1970 ◽

Vol 125 (5) ◽

pp. 825-829 ◽

Cited By ~ 6

Author(s):

S. G. Romeo

Keyword(s):

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Raynaud's Disease ◽

Raynaud’S Disease

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

VASA ◽

10.1024/0301-1526.34.4.243 ◽

2005 ◽

Vol 34 (4) ◽

pp. 243-249 ◽

Cited By ~ 2

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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Raynaud’s phenomenon and digital ischaemia - pharmacologic approach and alternative treatment options

VASA ◽

10.1024/0301-1526/a000526 ◽

2016 ◽

Vol 45 (3) ◽

pp. 201-212 ◽

Cited By ~ 13

Author(s):

Birgit Linnemann ◽

Matthias Erbe

Keyword(s):

Quality Of Life ◽

Angiotensin Receptor Blockers ◽

Raynaud’S Phenomenon ◽

Treatment Options ◽

Raynaud's Phenomenon ◽

Tissue Loss ◽

Receptor Antagonists ◽

Skin Ulcers ◽

Digital Ischaemia

Abstract. The primary goal of therapy is to reduce the frequency and intensity of Raynaud’s attacks and to minimize the related morbidity rather than to cure the underlying condition. Treatment strategies depend on whether Raynaud’s phenomenon (RP) is primary or secondary. All patients should be instructed about general measures to maintain body warmth and to avoid triggers of RP attacks. Pharmacologic intervention can be useful for patients with severe and frequent RP episodes that impair the patient’s quality of life. Calcium channel blockers are currently the most prescribed and studied medications for this purpose. There has been limited evidence for the efficacy of alpha-1-adrenergic receptor antagonists, angiotensin receptor blockers, topical nitrates or fluoxetine to treat RP. The intravenously administered prostacyclin analogue iloprost can reduce the frequency and severity of RP attacks and is considered a second-line therapy in patients with markedly impaired quality of life, critical digital ischaemia and skin ulcers who are at risk for substantial tissue loss and amputation. Phosphodiesterase inhibitors (e.g., sildenafil) can also improve RP symptoms and ulcer healing whereas endothelin-1 receptor antagonists (e.g., bosentan) are mainly considered treatment options in secondary prevention for patients with digital skin ulcers related to systemic sclerosis. However, their use in clinical practice has been limited by their high cost. Antiplatelet therapy with low-dose aspirin is recommended for all patients who suffer from secondary RP due to ischaemia caused by structural vessel damage. Anticoagulant therapy can be considered during the acute phase of digital ischaemia in patients with suspected vascular occlusive disease attributed to the occurrence of new thromboses. In patients with critical digital ischaemia, consideration should be given to hospitalisation, optimisation of medical treatment in accordance with the underlying disease and evaluation for a secondary, possibly reversible process that is causing or aggravating the clinical symptoms.

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