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Raynaud's disease and phenomenon [keywords]
- Therapeutic potentials of phosphodiesterase-5 inhibitors in cardiovascular disease. [Journal Article]
- Rev Cardiovasc Med 2014; 15(2):158-67.
Phosphodiesterase-5 (PDE5) inhibitors have been approved by the US Food and Drug Administration for the treatment of erectile dysfunction and more recently for pulmonary arterial hypertension (World Health Organization functional class I). PDE5 inhibitors can induce vasodilation; in addition, through a complex pathway involving nitric oxide, cyclic guanosine monophosphate, and protein kinase G, it can reduce apoptosis and suppress cell proliferation. The presence of PDE5 inhibitors in various tissues and systemic vasculature make them potential targets in a variety of cardiovascular diseases. In many in vitro and in vivo studies, PDE5 inhibitors have been shown to have positive effects in systolic and diastolic congestive heart failure, ischemic heart disease, doxorubicin cardiomyopathy, and pulmonary arterial hypertension. They also improved vasoconstriction in Raynaud phenomenon, peripheral artery disease, and hypoxic brain conditions. This article reviews the therapeutic potentials of PDE5 inhibitors in different cardiovascular diseases.
- Combined use of ursodeoxycholic acid and bosentan prevents liver toxicity caused by endothelin receptor antagonist bosentan monotherapy: two case reports. [JOURNAL ARTICLE]
- J Med Case Rep 2014 Jul 11; 8(1):250.
Pulmonary arterial hypertension is a fatal disease characterized by progressive remodeling of the pulmonary arteries and an increase in pulmonary vascular resistance. Up to 50% of patients with systemic sclerosis have pulmonary arterial hypertension, which significantly affects the prognosis. The endothelin receptor antagonist bosentan is used for the treatment of pulmonary arterial hypertension and shows a great beneficial effect. However, the most frequent side effect of bosentan is liver toxicity, which often requires dose reduction and discontinuation.We report two cases (a 64-year-old Japanese woman and a 69-year old Japanese woman) of systemic sclerosis, both with severe Raynaud's phenomenon and pulmonary arterial hypertension. Both patients had initially received bosentan monotherapy, which caused liver toxicity as indicated by increased levels of alanine aminotransferase, alkaline phosphatase, and gamma-glutamyltransferase. After dose reduction or discontinuation of bosentan, these liver function abnormalities were normalized and the patients subsequently received retreatment with a combination of bosentan and ursodeoxycholic acid. The results of liver function tests did not show any abnormalities after this combination therapy.These reports suggest the usefulness of ursodeoxycholic acid for preventing liver toxicity caused by bosentan. Thus, the addition of ursodeoxycholic acid to the treatment protocol is expected to be useful when liver toxicity emerges as a side effect of bosentan.
- Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. [JOURNAL ARTICLE]
- Clin Rheumatol 2014 Jul 4.
Because overlap syndromes (OSs) are rarely described, we analyzed retrospectively their frequencies and correlations in Brazilian series of 31 patients with dermatomyositis (DM)/polymyositis (PM) associated with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or rheumatoid arthritis (RA) attended at a referral single center. Myositis-specific autoantibodies (MSAs: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-SRP, anti-Mi-2) and myositis-associated autoantibodies (MAAs: anti-PM-Scl75, anti-PM-Scl100, anti-Ku) as well as specific autoantibodies related to SLE, SSc, and RA were investigated. The mean age of the OS patients (9 DM and 22 PM) was 44.6 ± 15.4 years, with a predominance of women (83.9 %) and white ethnicity (58.1 %). PM was the most frequent inflammatory myopathy, and the clinical presentation of DM/PM was significantly different among the OS groups. Overlap was found with SSc (48.4 %), SLE (29.0 %), and RA (22.6 %). The clinical manifestations of DM/PM were identified simultaneously with SSc and RA in the majority of cases, in contrast to identification in the SLE group (p < 0.05). All patients were positive for antinuclear antibodies, and the prevalence of MSA and MAA was 38.8 % in all OS groups, mutually exclusive, and more frequent in the SSc group. Comparing the clinical and laboratory features, there was a higher frequency of vascular (skin ulcers, Raynaud's phenomenon) and pulmonary (interstitial lung disease) involvement in the SSc group (p < 0.05). Moreover, there were no differences among the groups in relation to disease relapse and deaths. Concluding, this is the first study to show the different characteristics of a series of patients with connective tissue disease (CTD)-OS in the heterogeneous Brazilian population.
- Clinical Course, Prognosis, and Cause of Death in Primary Sjögren's Syndrome. [Journal Article]
- J Immunol Res 2014.:647507.
The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosed with primary Sjögren's syndrome (pSS) between 1975 and 2010. The patients were followed up for 11.4 ± 6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, and mortality ratios, and compared them within subgroups defined by gender, glandular and extraglandular manifestations (EGMs), associated diseases, and immunoserological abnormalities. The most frequent EGMs were polyarthritis, Raynaud's phenomenon, and vasculitis among our patients; the most common associated disease was thyroiditis. During the follow-up period, 51 patients died; the median survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was the most powerful predictor of mortality. Mortality in the group of patients with extraglandular symptoms was two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk.
- Critical acral ischemia leading to multiple finger amputation: Side effect of long-term (>30 cycles) pemetrexed maintenance treatment in a patient. [LETTER]
- Br J Clin Pharmacol 2014 Jun 24.
Pemetrexed is being used in United States (U.S.) for maintenance treatment of non-squamous non-small cell lung cancer for 4 years, after it was approved for this indication by U.S. Food and Drug Administration in July 2009. Here we report the first case of digital ischemia requiring amputation, likely due to long-term pemetrexed maintenance treatment. A 68-year-old female with lung adenocarcinoma developed painful fingers after 32 cycles of maintenance pemetrexed treatment. The signs and symptoms initially mimicked Raynaud's phenomenon, but rapid progression ensued to ischemic gangrene of the digits. Comprehensive blood work and imaging ruled out other systemic and vascular diseases. An emergent angiogram of upper extremity showed poor perfusion in distal digital arteries. Unfortunately, the gangrenous fingers needed to be amputated. Pemetrexed discontinuation resolved pain and ischemic symptoms in other fingers. This case illustrates that new-onset Raynaud's phenomenon during pemetrexed chemotherapy may portend a more ominous vascular condition that warrants further investigation when pain symptoms persist. We propose that cumulative toxicity from multiple pemetrexed infusions may lead to severe endothelial dysfunction and limb threatening gangrene. Post marketing surveillance of long-term pemetrexed (>30 cycles) should be studied to definitively determine a causal relationship between prolonged pemetrexed use and peripheral arterial ischemic disorders.
- Nailfold capillaroscopy in juvenile rheumatic diseases: known measures, patterns and indications. [JOURNAL ARTICLE]
- Clin Exp Rheumatol 2014 Jun 6.
Nailfold capillaroscopy has become an established method in adults for the evaluation of structural abnormalities of the microcirculation associated with rheumatic disease. It is a cornerstone for the diagnostic work-up of patients with Raynaud's phenomenon and the early diagnosis of systemic sclerosis. However, this non-invasive examination may also be valuable in children and adolescents with rheumatic diseases. Based on the scarce data available, this review focuses on capillaroscopic findings in healthy children and adolescents as well as in children with juvenile systemic sclerosis, juvenile dermatomyositis, juvenile idiopathic arthritis, and Raynaud's phenomenon. In addition, it outlines the potential benefits and limitations of nailfold capillaroscopy for routine care in paediatric rheumatology.
- [Nail-fold capillaroscopy in dermatology.] [JOURNAL ARTICLE]
- Ann Dermatol Venereol 2014 June - July; 141(6-7):429-437.
Nail-fold capillaroscopy is a non-invasive tool to study the microcirculation and is increasingly being used in dermatology, angiology and rheumatology. More recently, the use of video-capillaroscopy has allowed computer storage of capillaroscopic images (video-capillaroscopy), enabling evaluation of changes in capillaroscopic abnormalities during the follow-up of patients with systemic sclerosis or mixed connective tissue disease. Qualitative and quantitative assessment of the nail-fold dermal capillaries and of their organization can readily distinguish between a normal capillaroscopic pattern in primary Raynaud phenomenon and a specific sclerodermic pattern in secondary Raynaud phenomenon carrying a very high risk of systemic sclerosis. Apart from its important role as a diagnostic tool for distinguishing between primary and secondary Raynaud phenomenon, capillaroscopy is now used to predict the risk of development of digital ulcers and of future visceral complications in patients with systemic sclerosis. Moreover, nail-fold capillaroscopy is essential for differential diagnosis between connective tissue diseases, for the etiologic diagnosis of digital necrosis and diffuse interstitial lung disease, and in sclerodermiform syndromes.
- No association of atherosclerosis with digital ulcers in Japanese patients with systemic sclerosis: Evaluation of carotid intima-media thickness and plaque characteristics. [JOURNAL ARTICLE]
- J Dermatol 2014 Jun 18.
Patients with systemic sclerosis (SSc) usually develop Raynaud's phenomenon, persistent digital ischemia and sometimes develop digital ulcers (DU). Several studies have reported an association of carotid artery atherosclerosis with SSc by evaluating carotid intima-media thickness (IMT) in SSc patients. However, none of those studies analyzed the association between DU and carotid artery atherosclerosis in SSc patients. We examined the association of carotid artery atherosclerosis with digital ulcers by comparing SSc patients with (n = 48, 29.5%) and without (n = 206, 70.5%) DU. The demographic and clinical features of the SSc patients showed that young age, male sex, anti-topoisomerase I antibody positivity, severe skin sclerosis, interstitial lung disease complication and cardiac involvements were significantly prevalent in patients with DU. In addition, diffuse cutaneous type, anti-RNA polymerase III antibody positivity and severe skin sclerosis are more frequent in SSc patients with DU at the extensor surface of joints than SSc patients with DU at the digital tip. There were no differences in serum lipid level, carotid IMT or plaque score between SSc patients with and without DU, suggesting that atherosclerotic changes are not primarily involved in the development of DU.
- [Pharmacology of Raynaud's Phenomenon.] [JOURNAL ARTICLE]
- Therapie 2014 3-4; 69(2):115-128.
Raynaud's phenomenon (RP) is characterised by transient ischaemia in the extremities in response to cold or emotions. It can be primary (idiopathic) or secondary to an underlying disease. The pathophysiology of RP is multifactorial and complex. Microvascular impairment is a hallmark of the disease. The objective of this work is to review the different pharmacological treatments currently used in the management of RP, from their mechanism of action to the available evidence regarding their efficacy. We also propose to discuss potential pharmacological targets such as the potentiation of the nitric oxide pathway, or the inhibition of the RhoA-Rho kinase pathway. The last part of this review deals with drug-induced RP. Among various medications, beta-blockers, interferons, tyrosine-kinase inhibitors or cytotoxic agents such as bleomycin are involved.
- Conversion to Paradoxical Finding on Technetium-99m-labeled RBC Scintigraphy after Treatment for Secondary Raynaud's Phenomenon. [JOURNAL ARTICLE]
- Nucl Med Mol Imaging 2013 Dec; 47(4):278-280.
An 18-year-old woman reported that after exposure to cold temperatures her fingers appeared blue and her hands and feet felt cold. Secondary Raynaud's phenomenon (RP) associated with peripheral vascular disease was suspected. Technetium (Tc)-99m-labeled RBC hand scintigraphy after cold change showed decreased blood pool activity in her fingers. The patient's symptoms improved after she received sarpogrelate HCL (200 mg/day) and nifedifine (40 mg/day). Follow-up scintigraphy performed 7 months after the patient started treatment showed paradoxically increased blood pool activity in her fingers after cold challenge. To the best of our knowledge, this is the first case report of a patient with secondary RP showing paradoxical change on scintigraphy after she received medication that improved her symptoms.