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Raynaud's disease and phenomenon [keywords]
- Clinical differences between Thai systemic sclerosis patients with positive versus negative anti-topoisomerase I. [JOURNAL ARTICLE]
- Int J Rheum Dis 2014 Oct 8.
Anti-topoisomerase I antibody (ATA) carries an increased risk of systemic sclerosis (SSc) internal organ involvement. There have been no published comparisons of the clinical characteristics of patients positive and negative for ATA in Thailand, where the positive rate for ATA is higher than among Caucasians.To define the clinical differences between SSc, positive versus negative, for ATA.A retrospective cohort study was performed among SSc patients over 18 at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006-December 2013. SSc-overlap syndrome was excluded.Two hundred and ninety-four SSc patients were included (female : male 2.5 : 1). The majority (68.6%) were the diffuse cutaneous SSc subset (dcSSc). ATA was positive in 252 patients (85.7%), among whom 71.7% had dcSSc and 28.2% limited cutaneous SSc (lcSSc). Using a multivariate analysis, hand deformity had a significantly positive association with ATA (odds ratio [OR] 7.01; 95% CI 1.02-48.69), whereas being anti-centromere (ACA) positive had a negative association (OR 0.17; 95% CI 0.03-0.92). After doing a subgroup analysis of the SSc subset, the median duration of disease at time of pulmonary fibrosis detection among ATA positive dcSSc was significantly shorter than the ATA negative group (1.05 vs. 6.77 years, P = 0.01). Raynaud's phenomenon (RP) at onset was significantly more frequent in lcSSc sufferers who were ATA negative than those who were ATA positive (90.5% vs. 56.9%, P = 0.005).A high prevalence of ATA positivity was found among Thai SSc patients and this was associated with a high frequency of hand deformity, ACA negativity, a short duration of pulmonary fibrosis in dcSSc and a lower frequency of RP in lcSSc.
- Choroidal impairment and macular thinning in patients with systemic sclerosis: The acute study. [JOURNAL ARTICLE]
- Microvasc Res 2014 Sep 26.
Raynaud's phenomenon (RP) is a reversible vasospastic response of the extremities to cold or emotion, and can be the first manifestation or may be present before the development of an overt systemic sclerosis (SSc). The disturbance of the balance between vasodilation and vasoconstriction is not limited to the peripheral microcirculation of the skin, but it is also observed in other organs, such as the choroidal plexus of the eye. We aimed to examine the choroidal thickness (CT), the macular thickness and ganglion cell complex (GCC) average in thirty consecutive patients, without visual symptoms, classified as primary RP (pRP), RP secondary to suspected SSc, and overt SSc. All the patients underwent rheumatologic and ophthalmologic examination, capillaroscopy, test for anti-nuclear antibodies, anti-dsDNA, and anti-extractable nuclear antigens. Ophthalmologic examination included: best corrected visual acuity; slit lamp biomicroscopy; intraocular pressure measurements, fundus examination, and Spectral Domain-Optical Coherence Tomography (SD-OCT) with enhanced depth imaging scan system. Twenty-seven healthy subjects similar for gender and age were analyzed. In pRP, CT was significantly thinner than controls in the outer nasal and temporal regions. In secondary RP, the inner and outer nasal areas were significantly thinner than controls. In SSc, the central, inner inferior, inner nasal, inner superior, outer temporal, outer inferior, and outer nasal regions were significantly thinner than controls. A decreasing trend of central foveal thickness was noted. All the patients had GCC average significantly lower than controls. A thinning of choroidal and macular thickness, as well as of GCC was observed in patients with pRP and becomes more severe and extensive in RP secondary to suspected SSc and overt SSc. To our knowledge, this is the first study to analyze the choroidal features using SD-OCT in RP. These data may be clinically useful in suggesting an early involvement of ocular microcirculation with significant reduction of choroidal perfusion.
- The 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis Out-perform the 1980 Criteria. Data from the Canadian Scleroderma Research Group. [JOURNAL ARTICLE]
- Arthritis Care Res (Hoboken) 2014 Sep 18.
Objective: The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc) in an independent cohort of SSc subjects and to assess the contribution of individual items of the criteria to the overall sensitivity. Method: SSc subjects from the Canadian Scleroderma Research Group cohort were assessed. Sensitivity was determined in several subgroups of patients. In patients without the criterion of skin thickening proximal to the metacarpophalangeal joints (MCPs), we re-calculated sensitivity after removing individual criterion. Results: A total of 724 SSc patients were included. Most were females (86%), mean age was 55.8 years, mean disease duration was 10.9 years, and 59% had lcSSc. Overall, the sensitivity of the 2013 criteria was 98.3% compared to 88.3% for the 1980 criteria. This pattern was consistent among those with lcSSc (98.8% versus 85.6%), anti-centromere antibodies (98.9% vs 79.8%), disease duration ≤ 3 years (98.7% vs 84.7%) and no skin involvement proximal to the MCPs (97% vs 60%). In the latter sub-group, removing Raynaud's phenomenon and sclerodactyly from the criteria reduced the sensitivity to 77% and 79%, respectively. Removing both sclerodactyly and puffy fingers reduced the sensitivity to 62%. Conclusion: The 2013 SSc classification criteria classify more SSc patients than the 1980 criteria. The improvement in sensitivity is most striking in those with lcSSc, especially those without skin involvement proximal to the MCPs. The addition of Raynaud's phenomenon and puffy fingers to the 2013 criteria accounts for important gains in sensitivity. © 2014 American College of Rheumatology.
- Antiphosphatidylserine and antiphosphatidylethanolamine antibodies in systemic lupus erythematosus: occurrence and association with clinical disease manifestations. [JOURNAL ARTICLE]
- Pol Arch Med Wewn 2014 Jun 20.
INTRODUCTION Antiphosphatidylethanolamine antibodies (aPE) and antiphosphatidylserine antibodies (aPS) belong to a group of antiphospholipid antibodies (aPL) that occur in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). OBJECTIVES The aim of this study was to examine the relationship among the elevated serum concentration of aPE/aPS, the clinical manifestations of SLE, and the presence of other autoantibodies. PATIENTS AND METHODS The study group consisted of 71 SLE patients. The control group was made up of 36 healthy volunteers. In both groups aPS/aPE serum concentration was measured with enzyme immunoassays. Data, including clinical manifestations and the laboratory markers of SLE, were obtained from medical documentation. RESULTS The study revealed a higher prevalence of aPE in SLE patients than in the controls (54.93% vs. 5.56%). Compared with aPE, aPS occurred in the study group less frequently (12.68%) and were absent in the controls. Anticardiolipin antibodies (aCL) and APS were found to be related to aPS presence. Among aPS-positive SLE patients, thrombocytopenia, Raynaud's phenomenon, and myocardial infarction were observed more frequently. The study revealed that aPE presence predisposes the patient to mucosal ulcers. A positive correlation between aPS and ESR was also revealed. The serum concentration of aPE correlated negatively with red blood count (RBC) and positively with erythrocyte sedimentation rate (ESR). CONCLUSIONS The presence of aPS in SLE patients was found to be associated with thrombocytopenia, Raynaud's phenomenon, and cardiac complications.
- Partial fingertip necrosis following a digital surgical procedure in a patient with primary Raynaud's phenomenon. [JOURNAL ARTICLE]
- Int Wound J 2014 Sep 9.
Raynaud's phenomenon is a common clinical disorder consisting of recurrent, long-lasting and episodic vasospasm of the fingers and toes often associated with exposure to cold. In this article, we present a case of partial fingertip necrosis following digital surgical procedure in a patient with primary Raynaud's phenomenon.
- Guillain-Barré syndrome presenting with Raynaud's phenomenon: a case report. [Journal Article]
- BMC Neurol 2014; 14(1):174.
Guillain-Barré syndrome is an immune mediated acute inflammatory polyradiculo-neuropathy involving the peripheral nervous system. Commonest presentation is acute or subacute flaccid ascending paralysis of limbs. Rarely autonomic dysfunction can be the presenting feature of Guillain-Barré syndrome. Raynaud's phenomenon, although had been described in relation to many disease conditions, has not been described in association with Guillain-Barré syndrome up to date.We report the first case of Guillain-Barré syndrome presenting with Raynaud's phenomenon in a 21-year-old previously well boy. New onset Raynaud's phenomenon was experienced followed by acute ascending flaccid paralysis of lower limbs and upper limbs together with palpitations and postural giddiness. Nerve conduction studies showed acute inflammatory demyelinating polyneuropathy with cerebrospinal fluid cyto-protein dissociation. He was treated with intravenous immunoglobulin and showed a satisfactory clinical recovery of muscle weakness, Raynaud's phenomenon and autonomic disturbances.Guillain-Barré syndrome presenting with Raynaud's phenomenon is not being reported in literature previously. Although the underlying mechanism is not fully understood, Raynaud's phenomenon should prompt the physician to consider Guillain-Barré syndrome with a complimentary clinical picture.
- Postural orthostatic tachycardia syndrome: a dermatologic perspective and successful treatment with losartan. [Journal Article]
- J Clin Aesthet Dermatol 2014 Aug; 7(8):41-7.
The postural orthostatic tachycardia syndrome is a disease characterized by excessively increased heart rate during orthostatic challenge associated with symptoms of orthostatic intolerance including dizziness, exercise intolerance, headache, fatigue, memory problems, nausea, blurred vision, pallor, and sweating, which improve with recumbence. Postural orthostatic tachycardia syndrome patients may present with a multitude of additional symptoms that are attributable to vascular vasoconstriction. Observed signs and symptoms in a patient with postural orthostatic tachycardia syndrome include tachycardia at rest, exaggerated heart rate increase with upright position and exercise, crushing chest pain, tremor, syncope, loss of vision, confusion, migraines, fatigue, heat intolerance, parasthesia, dysesthesia, allodynia, altered traditional senses, and thermoregulatory abnormalities. There are a number of possible dermatological manifestations of postural orthostatic tachycardia syndrome easily explained by its recently discovered pathophysiology. The author reports the case of a 22-year-old woman with moderate-to-severe postural orthostatic tachycardia syndrome with numerous dermatological manifestations attributable to the disease process. The cutaneous manifestations observed in this patient are diverse and most noticeable during postural orthostatic tachycardia syndrome flares. The most distinct are evanescent, hyperemic, sharply demarcated, irregular patches on the chest and neck area that resolve upon diascopy. This distinct "evanescent hyperemia" disappears spontaneously after seconds to minutes and reappears unexpectedly. Other observed dermatological manifestations of this systemic disease include Raynaud's phenomenon, koilonychia, onychodystrophy, madarosis, dysesthesia, allodynia, telogen effluvium, increased capillary refill time, and livedo reticularis. The treatment of this disease poses a great challenge. The author reports the unprecedented use of an oral angiotensin II type 1 receptor antagonist resulting in remarkable improvement.
- The association between vibration and vascular injury in rheumatic diseases: A review of the literature. [JOURNAL ARTICLE]
- Autoimmunity 2014 Aug 12.:1-8.
Abstract Vascular manifestations can be seen early in the pathogenesis of inflammatory rheumatic diseases. Animal experiments, laboratory and clinical findings indicated that acute or long-term vibration exposure can induce vascular abnormalities. Recent years, in addition to Raynaud's phenomenon (RP), vibration as a risk factor for other rheumatic diseases has also received corresponding considered. This review is concentrated upon the role of vibration in the disease of systemic sclerosis (SSc). In this review, we are going to discuss the main mechanisms which are thought to be important in pathophysiology of vascular injury under the three broad headings of "vascular", "neural" and "intravascular". Aspects on the vibration and vascular inflammation are briefly discussed. And the epidemiological studies related to vibration studies in SSc and other rheumatic diseases are taken into account.
- Effectiveness of calcium channel blockers for Raynaud phenomenon. [Journal Article, Meta-Analysis]
- Am Fam Physician 2014 Aug 1; 90(3):143-4.
- IgG4-related skin disease. [JOURNAL ARTICLE]
- Br J Dermatol 2014 Jul 26.
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a recently established clinical entity characterized by high levels of circulating IgG4 and tissue infiltration of IgG4(+) plasma cells. IgG4-RD exhibits a distinctive fibro-inflammatory change involving multiple organs, such as pancreas, and salivary and lacrimal glands. The skin lesions of IgG4-RD have been poorly characterized and may stem from not only direct infiltration of plasma cells but also IgG4-mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek, and mandible regions), (3) Mikulicz's disease (palpebral swelling, sicca syndrome, and exophthalmos), (4) psoriasis-like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura), and (7) ischemic digit (Raynaud's phenomenon and digital gangrene). It is considered that the subtypes (1)-(3) are induced by direct infiltration of IgG4(+) plasma cells, while the other (4)-(7) types are caused by the secondary mechanisms. IgG4-related skin disease is defined as IgG4(+) plasma cell-infiltrating skin lesions that form plaques, nodules or tumors (1-3), but may manifest secondary lesions caused by IgG4+ plasma cells and/or IgG4 (4-7). This article is protected by copyright. All rights reserved.