- Raynaud's Phenomenon: A Brief Review of the Underlying Mechanisms. [Review]
- FPFront Pharmacol 2016; 7:438
- Raynaud's phenomenon (RP) is characterized by exaggerated cold-induced vasoconstriction. This augmented vasoconstriction occurs by virtue of a reflex response to cooling via the sympathetic nervous s...
Raynaud's phenomenon (RP) is characterized by exaggerated cold-induced vasoconstriction. This augmented vasoconstriction occurs by virtue of a reflex response to cooling via the sympathetic nervous system as well as by local activation of α2C adrenoceptors (α2C-AR). In a cold-initiated, mitochondrion-mediated mechanism involving reactive oxygen species and the Rho/ROCK pathway, cytoskeletal rearrangement in vascular smooth muscle cells orchestrates the translocation of α2C-AR to the cell membrane, where this receptor readily interacts with its ligand. Different parameters are involved in this spatial and functional rescue of α2C-AR. Of notable relevance is the female hormone, 17β-estradiol, or estrogen. This is consistent with the high prevalence of RP in premenopausal women compared to age-matched males. In addition to dissecting the role of these various players, the contribution of pollution as well as genetic background to the onset and prevalence of RP are also discussed. Different therapeutic approaches employed as treatment modalities for this disease are also highlighted and analyzed. The lack of an appropriate animal model for RP mandates that more efforts be undertaken in order to better understand and eventually treat this disease. Although several lines of treatment are utilized, it is important to note that precaution is often effective in reducing severity or frequency of RP attacks.
- Myeloproliferative Disease: An Unusual Cause of Raynaud's Phenomenon and Digital Ischaemia. [Journal Article]
- CRCase Rep Med 2016; 2016:9675171
- We describe a 59-year-old female who presented with ischaemic digits, preceded by a 6-month history of Raynaud's phenomenon affecting her fingers and toes. There were no clinical or laboratory featur...
We describe a 59-year-old female who presented with ischaemic digits, preceded by a 6-month history of Raynaud's phenomenon affecting her fingers and toes. There were no clinical or laboratory features of primary vasculitis or connective tissue disease, Doppler imaging was normal, and bloods were unremarkable aside from a platelet count of 786 × 109/L (150-400) and white cells of 16 × 109/L (4-11). In view of the thrombocytosis a JAK2 mutation assay was requested which confirmed a JAK2 V617F mutation, suggesting essential thrombocytosis (ET) as the cause. She received treatment with hydroxycarbamide which normalised her platelet count and led to a complete resolution of her Raynaud's symptoms. Raynaud's phenomenon is a rare manifestation of ET. Myeloproliferative disorders such as ET should be considered in the differential diagnosis of Raynaud's phenomenon and vasculitis.
- Myocardial perfusion in peripheral Raynaud's phenomenon. Evaluation using stress cardiovascular magnetic resonance. [Journal Article]
- IJInt J Cardiol 2016 Nov 12; 228:444-448
- CONCLUSIONS: MPRI reduction is common in both PRP and SRP, but it is more severe in SRP, even if RP patients are under treatment with calcium blockers. Occult fibrosis may coexist with the reduced MPRI in SRP but not in PRP.
- The 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis Could Classify Systemic Sclerosis Patients at Earlier Stage: Data from a Chinese EUSTAR Center. [Journal Article]
- PlosPLoS One 2016; 11(11):e0166629
- CONCLUSIONS: Relative to the 1980 ACR criteria, in Chinese SSc patients the new 2013 ACR/EULAR criteria had similar specificity and higher sensitivity, especially for patients with mild skin thickening or prominent microvascular diseases.
- Systematic review of systemic sclerosis-specific instruments for the EULAR Outcome Measures Library: An evolutional database model of validated patient-reported outcomes. [Journal Article]
- SASemin Arthritis Rheum 2016 Oct 13
- CONCLUSIONS: Seven SSc-specific PROs have a minimum validation and were included in the EULAR OML. Further development in the area of disease-specific PROs in SSc is warranted.
- A literature review of eosinophilic fasciitis with an illustrated case. [Journal Article]
- CRCurr Rheumatol Rev 2016 Oct 07
- Eosinophilic fasciitis (EF), a rare connective tissue disorder, was first reported by Lawrence Shulman in 1974. Since then over 300 cases have been reported worldwide. EF has variable clinical presen...
Eosinophilic fasciitis (EF), a rare connective tissue disorder, was first reported by Lawrence Shulman in 1974. Since then over 300 cases have been reported worldwide. EF has variable clinical presentations and currently does not have internationally accepted diagnostic criteria. Dermatological features are the most ubiquitously present symptoms. It often presents with scleroderma-like skin changes. Extracutaneous presentations, such as arthritis and carpal tunnel syndrome can precede cutaneous changes. The most useful clinical features are the lack of Raynaud's phenomenon, telangiectasia and visceral involvement, differentiating it from Scleroderma. Haematological disorders, solid tumours and autoimmune disorders are frequently associated with EF. Historically, the presence of peripheral eosinophilia, elevated ESR and hypergammaglobulinemia were considered to be diagnostic of EF. It is now well recognised that neither the presence of eosinophilia in the blood nor eosinophilic infiltartion in the fascia is necessary for its diagnosis. An en bloc surgical biopsy including skin, subcutis, fascia and muscle is the gold-standard test for diagnosing EF. Magnetic resonance imaging helps to locate a suitable biopsy site and to monitor treatment response. Although its underlying aetiology is unknown, there is growing evidence supporting an immunological origin. Immunosuppressive drugs are used to treat EF and the corticosteroid is the first line treatment. A significant proportion of patients can develop permanent disabilities such as joint contractures, tendon retraction and subdermal sclerosis. Occasionally it can be treatment refractory or have a relapse-remitting course. We report another case of EF with a literature review of epidemiology, disease manifestations, aetiology, investigations, treatment and prognosis.
- Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus. [Journal Article]
- LLupus 2016 Nov 10
- CONCLUSIONS: Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.
- Neonatal Behçet's disease with Raynaud phenomenon. [Journal Article]
- JNJ Neonatal Perinatal Med 2016 Nov 9
- We report a new clinical manifestation of neonatal Behcet's disease. A newborn from a mother with active Behcet's disease during pregnancy, who develops vasomotor phenomena (Raynaud) with oral ulcera...
We report a new clinical manifestation of neonatal Behcet's disease. A newborn from a mother with active Behcet's disease during pregnancy, who develops vasomotor phenomena (Raynaud) with oral ulcerations in the second day of life. Neonatal mucocutaneous lesions have been reported previously in few newborns of pregnant women with active Behçet disease. Although neonatal disease is a very infrequent situation, with a mostly transient and favorable course, clinicians should be aware of serious potential complications associated with this entity.
- Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc. [Journal Article]
- MRMod Rheumatol 2016 Nov 14; :1-4
- CONCLUSIONS: The new 2013 criteria can classify most mild/early Japanese SSc patients, which may contribute to early treatment interventions.
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- Syndrome in question: antisynthetase syndrome (anti-PL-7). [Case Reports]
- ABAn Bras Dermatol 2016 Sep-Oct; 91(5):683-685
- Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12...
Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.