Association of Clinical Manifestations of Systemic Lupus Erythematosus and Complementary Therapy Use in Taiwanese Female Patients: A Cross-Sectional Study.

Background and Objectives: Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease that affects predominantly women in the childbearing years. Patients may seek complementary therapies to manage their health and to reduce symptoms. However, to our knowledge, no studies have explored the association between clinical manifestations of SLE and complementary therapies. Therefore, this study aimed to investigate the association of complementary therapies with common clinical manifestations in Taiwanese female patients with SLE. Materials and Methods: A cross-sectional study was conducted at a regional teaching hospital in southern Taiwan. Outpatients from the rheumatology clinic who met the inclusion criteria were consecutively recruited. Demographic data, clinical manifestations of SLE, and types of complementary therapy use were determined using paper-based questionnaire. Multiple logistic regression analyses were conducted to investigate the use of complementary therapies associated with clinical manifestations of SLE. Results: Of the 317 female patients with SLE, 60.9% were 40 years or older. The five SLE clinical manifestations with the highest prevalence were Raynaud's phenomenon (61.2%), photosensitivity (50.2%), Sjögren's syndrome (28.4%), arthralgia and arthritis (22.1%), and renal involvement (14.5%). Multiple logistic regression analyses revealed that Raynaud's phenomenon was significantly associated with fitness walking or strolling (adjusted odds ratio [aOR] 1.77; p = 0.027) and fish oil supplements (aOR 3.55, p < 0.001). Photosensitivity was significantly and inversely associated with the use of probiotics (aOR 0.49; p = 0.019). Renal involvement was significantly associated with the use of probiotics (aOR 2.43; p = 0.026) and visit to the Chinese medicine department in a hospital (aOR 3.14, p = 0.026). Conclusions: We found that different clinical manifestations of SLE were associated with the use of different complementary therapies. Health care providers should have up-to-date knowledge of common complementary therapies and be ready to provide evidence-based advice to patients with SLE.

Treatment of Raynaud phenomenon and ischemic ulcers associated to systemic sclerosis with hyperbaric oxygen.

We describe 4 patients with Raynaud's phenomenon associated with systemic sclerosis, 3 with ischaemic ulcers, successfully treated with hyperbaric oxygen. This therapy has been useful in the treatment of chronic wounds due to its anti-inflammatory, antimicrobial and angiogenic effects. Hyperbaric oxygen treatment could be a therapeutic option in patients with Raynaud's phenomenon refractory to conventional treatment.

What is the Best Way to Treat Patients with Raynaud's Phenomenon and a Tendency towards Hypotension?

Episodes of excessive vasospasm are common in patients with Raynaud's phenomenon (RP). Pharmacological treatment may often result in side-effects such as hypotension, leading to discontinuation of treatment. Review of therapeutic interventions with regard to tendency towards hypotension was done in medical databases including PubMed, Scopus, and Medline to summarize the current state of the knowledge. Despite the episodes of blood pressure drops caused by hypotension, calcium channel blockers (CCB) have been widely used in RP as first-line treatment medication. The use of other CCB apart from nifedipine is controversial due to the variety of results in clinical trials. A clinical study comparing the efficacy and tolerability of losartan with nifedipine revealed a significant reduction in RP severity, frequency of episodes, and reported adverse effects. Application of oral sildenafil 100 mg/d as an add-on therapy increased microvascular blood flow in secondary RP, while being well-tolerated and with no withdrawal from the study. Topical vasodilators may be applied as an adjuvant therapy for patients with RP. Clinical studies approved 10% nifedipine cream and 10% nitroglycerine gel as an efficient RP therapy with side-effects comparable with placebo usage. Non-pharmacological interventions, such as cold avoidance, stress management, and smoking cessation are recommended in reducing episodes of RP. Calcium channel blockers, with a particular emphasis on nifedipine, in combination with non-pharmacological management seem to be the optimal way to treat the patients with a tendency to hypotension.

Raynaud Phenomenon of the Nipple: An Under-Recognized Condition.

BACKGROUND: Obstetricians often feel ill-equipped to address the symptom of breast pain in pregnant and postpartum patients. CASES: In the first case, a 40-year-old woman in the second trimester of pregnancy reported nipple discoloration and severe pain. She was treated with nifedipine, and her symptoms decreased quickly and markedly. In the second case, a 32-year-old woman presented for a routine postpartum visit. She described breast pain and sporadic purple discoloration of the nipples, a finding confirmed on examination. Conservative measures of maintaining warmth were recommended. CONCLUSION: Raynaud phenomenon of the nipple is an underdiagnosed condition affecting women in both the prenatal and postpartum periods. A careful history and physical must be obtained in women presenting with breast pain, because diagnosis and treatment allows breastfeeding continuation and mitigation of symptoms.

Raynaud's phenomenon: Current concepts.

Raynaud's phenomenon (RP) is a transient, acral, vasospastic phenomenon that manifests with characteristic color changes. This vasospasm, classically triggered by cold temperatures, may also be driven by shifts in temperature, climate, or emotional state. Primary RP (PRP) is a common condition without severe sequelae. Secondary RP (SRP), which may be driven by vascular, autoimmune, hematologic, or endocrine etiologies, can result in digital ulceration, irreversible ischemia and necrosis, and secondary infection. This review delineates the clinical manifestations of both primary and secondary RP, as well as the current understanding of RP epidemiology and pathogenesis. Proper examination, including nailfold capillary microscopy, and laboratory workup for secondary causes of RP are also discussed. The traditional armamentarium of therapies used for RP, as well as newer medical and surgical options, is also summarized with particular regard to the clinical evidence for their efficacy.

Difficulties in diagnosis and treatment of severe secondary Raynaud's phenomenon in a Cameroonian woman: a case report.

BACKGROUND: Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment. CASE PRESENTATION: A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief. CONCLUSIONS: An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading.

Esclerodermia y paniculitis localizadas / Localized scleroderma and panniculitis

La esclerodermia es una enfermedad del tejido conectivo, autoinmunitaria y caracterizada por fibrosis de la piel1-3. Literalmente significa 'piel dura'. La afectación puede ser mínima (solo en dedos y cara, muy lentamente progresiva) o generalizada (afectando de forma rápida a uno o más órganos internos). La paniculitis neutrofílica4,6 es una respuesta inmunitaria localizada en forma de placa o nódulo, en el seno generalmente de una enfermedad sistémica (AU)

Scleroderma is a disease that affects the connective tissue. It is an autoimmune disease and it is characterized by skin fibrosis. It literally means 'hard skin'. The involvement can be minimal (only fingers and face, progressing slowly) or generalized (quickly affecting one or two internal organs). Neutrophilic panniculitis is an immune response located in the form of a plate or nodule, generally related to a systemic disease (AU)

Coexistence of osteopoikilosis with seronegative spondyloarthritis and Raynaud's phenomenon: first case report with evaluation of the nailfold capillary bed and literature review.

Osteopoikilosis (OPK) is a rare autosomal dominant bone disorder characterized by numerous hyperostotic areas that tend to localize in periarticular osseous regions. It is usually asymptomatic and is often diagnosed incidentally during X-rays. OPK may be an isolated finding or associated with other pathologies, e.g. skin manifestations, rheumatic and/or skeletal disorders. We report a literature review and, for the first time, the coexistence of OPK with seronegative spondyloarthritis and Raynaud's phenomenon in a 48-year old female. To the best of our knowledge, this is the first case of OPK studied by videocapillaroscopy, demonstrating the absence of specific microvascular abnormalities of nailfold capillaries.

Fenómeno de Raynaud y el amamantamiento doloroso / Raynaud's phenomenon and pain during breastfeeding

El fenómeno de Raynaud es una patología provocada por la exposición al frío, que conduce al vasoespasmo intermitente de arteriolas, produciendo un cuadro clínico por la falta de aporte de oxígeno, consistente en palidez, parestesias y dolor de la zona afectada1. Es una causa tratable de amamantamiento doloroso que llega a afectar a 1 de cada 5 mujeres en edad fértil y que en ocasiones lleva al abandono de la lactancia si es erróneamente diagnosticado por su incomoda sintomatología (AU)

Raynaud's phenomenon of the nipple is a pathology precipitated by exposure to cold and leads to intermittent vasospasm of arterioles, producing clinical symptoms of pain, burning and paresthesias. This condition is a treatable cause of painful breastfeeding that can affect up to 1 in 5 women of childbearing age. If misdiagnosed, it can occasionally lead to cessation of breastfeeding due to its uncomfortable symptomatology (AU)

Syncope and Raynaud's disease.

OBJECTIVE: To investigate an association between syncope and Raynaud's disease (RD), its clinical features, and the effect of treatment with nifedipine. DESIGN: One-year prospective study of new outpatients after 3 initial clinical observations. SETTING: Neurology clinics at Chelsea and Westminster, Royal Free, Barnet, and Edgware Hospitals. PATIENTS: Ten women and 1 man. The group had a mean (SD) age of 33 (17) years. Mean (SD) follow-up was 24 (36) months. INTERVENTION: Treatment with nifedipine. OUTCOME MEASURES: Observed vs expected frequency of syncope in RD, temporal relation between syncope and Raynaud's phenomenon, clinical features, and response to nifedipine treatment. RESULTS: Eight additional patients with syncope and RD were identified from 603 new patients (1.3%); we had expected only 1 patient to be identified with syncope and RD (P=.003). A chance association between RD and migraine with recurrent syncope was unlikely (P=.01). The prevalence of RD in patients with syncope with migraine was higher than expected (P=.03), but that of migraine in patients with RD was not (P=.2). All 11 patients had 5 or more syncopal episodes for a median of 2 years (range, 0.1-62 years). Three patients had previous diagnoses of nonepileptic attacks. Syncope was preceded by or contemporaneous with Raynaud's phenomenon in 10 patients (P=.02). Nine patients had migraine; headache was contemporaneous with syncope in 4 patients as expected by chance (P=1.0). In all patients, syncope was preceded by brainstem or vertebrobasilar symptoms, and it ceased after treatment with nifedipine. Raynaud's disease and migraine improved less. CONCLUSIONS: The association of syncope to RD was unrelated to chance or migraine. The temporal relation between syncope and Raynaud's phenomenon but not headache was statistically significant. Treatment with nifedipine stopped recurrent syncope in all patients. Syncope related to RD may result from brainstem ischemia. Unexplained recurrent syncope should prompt screening for RD.

Spinal cord stimulation for Raynaud's syndrome: long-term alleviation of bilateral pain with a single cervical lead.

INTRODUCTION: Spinal cord stimulation (SCS) has been described in a variety of neuropathic and vasospastic pain conditions including Raynaud's syndrome. METHODS: We report here the outcome of single lead SCS in the case of a 49-year-old woman with severe Raynaud's syndrome, which had failed to respond to medical therapy. RESULTS: With a single quadripolar cervical lead in midline position at the C2/C3 level sustained pain relief of the bilateral pain was accomplished. Pain scores sank from 7/10 to 2-3/10 on the nominal analog scale and remained stable more than nearly four years by now. CONCLUSIONS: Treatment of bilateral pain in Raynaud's syndrome with SCS in a single technique is feasible. Advantages and disadvantages as compared with stimulation with bilateral leads are discussed.

25. Ischemic pain in the extremities and Raynaud's phenomenon.

Two important groups of disorders result from an insufficient blood supply to the extremities: critical vascular disease and the Raynaud's phenomenon. The latter can be subdivided into a primary and a secondary type. Critical ischemic disease is often caused by arteriosclerosis due to hypertension or diabetes. Primary Raynaud's is idiopathic and will be diagnosed as such if underlying systemic pathology has been excluded. Secondary Raynaud's is often a manifestation of a systemic disease. It is essential to try to establish a diagnosis as soon as possible in order to influence the evolution of the disease. A sympathetic nerve block can be considered in patients with critical ischemic vascular disease after extensive conservative treatment, preferably in the context of a study (2B±). If this has insufficient effect, spinal cord stimulation can be considered in a selected patient group (2B±). In view of the degree of invasiveness and the costs involved, this treatment should preferably be applied in the context of a study and with the use of transcutaneous pO(2) measurements. In case of primary Raynaud's, life style changes are the first step. Sympathectomy can be considered as a treatment of Raynaud's phenomenon (2C+), but only after multidisciplinary evaluation of the patient and in close consultation with the patient's rheumatologist, vascular surgeon or internist.

Peripheral blood perfusion correlates with microvascular abnormalities in systemic sclerosis: a laser-Doppler and nailfold videocapillaroscopy study.

OBJECTIVE: To investigate possible correlations between fingertip blood perfusion (FBP) status, assessed by laser Doppler flowmetry (LDF), and morphological microvascular abnormalities, detected by nailfold videocapillaroscopy (NVC), in patients with systemic sclerosis (SSc). The effects on FBP of intravenous (IV) treatment with the prostacyclin analog iloprost were also investigated. METHODS: Thirty-four consecutive patients with SSc and 16 healthy subjects were evaluated. LDF was performed by analyzing blood perfusion at the fingertips in both hands. Patients with SSc were distributed into the appropriate NVC pattern of microangiopathy (early, active, and late). Iloprost was administered to inpatients with SSc by 24-hour IV infusion for 7 consecutive days (4 microg/h). RESULTS: FBP was significantly lower in patients with SSc (p < 0.05) compared to controls. Heating of the LDF probe at 36 degrees C induced a significant increase of FBP in all subjects (p < 0.001), but the slope of variation was significantly lower in patients with SSc compared to controls (p < 0.05). Patients with SSc showing the late NVC pattern of microangiopathy had significantly lower FBP than patients with the active and early NVC patterns (p < 0.05). A negative correlation was observed between FBP and NVC rating of the microvascular damage (p < 0.05). After iloprost treatment, a significant increase of FBP was observed in patients with SSc (p < 0.05). CONCLUSION: Patients with SSc show a decreased FBP partially reversible by local skin heating. The FBP correlated negatively with the extent of nailfold microvascular damage, and IV iloprost treatment increased the FBP.

Percutaneous upper thoracic radiofrequency sympathectomy in Raynaud phenomenon: a comparison of T2/T3 procedure versus T2 lesion with phenol application.

BACKGROUND AND OBJECTIVES: Percutaneous radiofrequency (RF) thoracic sympathectomy is an alternative method to surgical procedures for the treatment of acral ischemia in Raynaud phenomenon. The procedure is indicated if conservative therapy fails to provide sufficient relief. The aim of this study was to compare classic T2 and T3 RF thermolesioning with a less invasive procedure at the level of T2 only. METHODS: Fifty adult patients, American Society of Anesthesiologists (ASA) classification I to III, were randomly assigned to 1 of 2 groups. T2 and T3 thoracic RF thermolesion was performed in 1 group, whereas T2 thermolesion with local application of 0.5 mL of 6% phenol was delivered in the second group. Changes in cold perception, pain, and quality of life were assessed using a questionnaire. Blood circulation in the upper extremity was evaluated using infrared thermography. Patients were observed for a period of 3 months. RESULTS: A significant decrease in pain according to visual analog scale (P < 0.001), increase in peripheral temperature in the upper extremities (P < 0.001), and improvement in quality of life were observed in both groups of patients after the procedure. Susceptibility to cold-provoked vasospasm was not significantly affected in either group. There was no significant difference between the 2 groups in any parameter apart from the duration of the procedure. CONCLUSIONS: Thoracic RF upper sympathectomy is an effective method in the treatment of resistant forms of Raynaud phenomenon. A single-shot procedure at the level of T2 may be preferable because of the shorter procedure duration of this technique.

Fenómeno de Raynaud / Raynaud’s Phenomenon

El fenómeno de Raynaud se caracteriza por una disminución de la circulación sanguínea, fundamentalmenteen los dedos de las manos y de los pies, con dolor y cambios de coloración en la piel deuna forma secuencial: blanco, azul y rojo. Es más frecuente en las mujeres, en la 2ª y 3ª décadasde la vida. Se desconoce cuál es su causa, pero guarda relación con el consumo de tabaco, alcohol,y fundamentalmente con los cambios de temperatura y alteraciones emocionales. Se asocia aenfermedades del tejido conectivo, entre las que Esclerodermia y Lupus son las más frecuentes. ElNifedipino es el fármaco de primera elección(AU)

Raynaud’s phenomenon is characterized by a decrease in blood circulation mainly in the fi ngers andtoes, with pain and changes in skin colour which follow a characteristic pattern white, blue and red. Itis more common in women, in the 2nd and 3rd decade of life. The cause is unknown, but is related tosmoking and alcohol consumption, and particularly body temperature changes and emotional stress.It is associated with connective tissue diseases amongst which scleroderma and lupus are the mostfrequent. Nifedipine is the treatment of choice(AU)

[The treatment of neurospastic vascular diseases in Raynaud phenomenon].

The efficacy of proposed methods for cardiovascular diseases with Raynaud phenomenon manifestations treatment was estimated. After application of the author method the patients extremities temperature had raised by 2 .50 degreesC. They had noted the pain and the extremities oedema elimination, trophic ulcers healing. According to Doppler investigation data the blood flow linear speed increase by 30 0% in comparison with pretreatment one was noted. The posttreatment complications were absent. Fair and good results were observed in 306 (82.3%) patients, in 21 (5.6%) patients the treatment was repeated in conditions of severe ischemia and the blood flow decompensation present. In 14 (3.8%) patients a high epidural blockade of sympathic nodes in portion of high thoracic and lower cervical vertebra according to the author method was performed. In far-remote period (in 3-10 years) the positive effect was prolonged in 298 (80.1%) patients.

Estimulación Medular (EEM) en el tratamiento de la enfermedad vascular periférica. Parte I / Spinal cord Stimulation for peripheral vascular disease treatment

Las técnicas de neuromodulación suponen un abordaje no destructivo y reversible en el tratamiento del dolor. Su utilización se establece como estrategia de control del dolor no controlable dentro de las directrices de la O.M.S. y como alternativa a otras terapias invasivas menores o neuroablatibas que no han sido eficaces o que están contraindicadas. De entre ellas el uso clínico de la estimulación eléctrica medular (EEM) o neuroestimulación, demuestra día a día su utilidad y eficacia en el tratamiento de diferentes síndromes de dolor crónico. Estudios de Cook y cols en 1.976, ya demostraron en sus trabajos la efectividad de esta técnica en la manifestación clínica del dolor isquémico así como en la arterioesclerosis o vasculopatía diabética, o en el dolor isquémico producido por vaso espasmo periférico, en entidades específicas como la Enfermedad de Buerguer, Esclerodermia o Síndrome de Raynaud. Durante la última década, se ha extendido la utilización de la EEM en la cardiopatía isquémica de carácter intratable (angina de pecho refractaria a tratamientos, síndrome “X”,...) y la patología vascular periférica, objeto de esta revisión. Esta técnica se presenta como una de las principales indicaciones con altos niveles de efectividad (80%), tanto en el aspecto clínico por el alivio sintomático que produce, como en los beneficios isquémicos fisiopatológicos que induce la neuroestimulación (AU)

The Neuromodulation technologies are a not destructive and reversible approach in the treatment of the pain. They are used as a strategy for the control of untreatable pain according to the directives of the OMS and as an alternative of other invasive minor or neuroablatives therapies that have been not effective or that are not indicated. Between them the clinical use of spinal cord stimulation (SCS) has demonstrated its usefulness and efficiency in the treatment of different syndromes of chronic pain. Cook´s and cols. in 1.976, demonstrated in their reports the efficiency of this technique in the clinical manifestation of ischemic pain as well as in the arteriosclerosis, diabetic vasculopaty, or in the ischemic pain related with peripheral vasospasm, in specific pathologies as the Buerguer´s disease, Scleroderma or Raynaud´s syndrome. During the last decade, the utilization of the SCS has spread in the ischemic cardiopathy of untreatable character (refractory angina pectoris, syndrome “X”,…) and vascular peripheral pathology, subject of this review. This technique appears as one of the main indications with high levels of efficiency (80%), so much in the clinical aspect for the symptomatic relief as well as in the ischemic physiopatologic benefits that induces the neuroestimulation (AU)