1,721,108 research outputs found
Etiopathogenesis of Systemic Sclerosis: Role of Human Parvovirus B19
No full textSystemic sclerosis, one of the most obscure rheumatic autoimmune diseases, is characterized by endothelial dysfunction and activation of fibroblasts, leading to diffuse microangiopathy and fibrosis of skin and visceral organs. Parvovirus B19,the virus responsable of the Fifth Disease, may infect also endothalial cells and their bone marrow precursors, along with fibroblasts; moreover, it has been associated to several autoimmune disorders, among which also systemic sclerosis. Is there a role for the virus in the etiopathogenesis of systemic sclerosis
Viral infections and systemic sclerosis.
No full textnon presente in quanto trattasi di Lettera all'editor
Vasculiti ANCA associate
No full textINTRODUZIONE. Le vasculiti associate agli anticorpi contro proteine dei granuli citoplasmatici dei neutrofili (ANCA) fanno parte delle cosiddette vasculiti sistemiche, un gruppo di condizioni morbose caratterizzate dalla presenza di flogosi e necrosi della parete vascolare e da una marcata eterogeneità dal punto di vista eziopatogenetico, clinico e prognostico. In generale, nelle vasculiti, il processo infiammatorio può essere a carico di arterie di vario calibro e può coinvolgere anche le vene ed i piccoli vasi (arteriole, capillari e venule). La vasculite è la risultante di un processo immuno-mediato a cui partecipano sia cellule dell’immunità innata (macrofagi, neutrofili, eosinofili) sia i linfociti B e T; inoltre, un ruolo importante è rivestito anche da fattori circolanti quali autoanticorpi, immunocomplessi, complemento e numerosi mediatori della flogosi.
Vi è un certo overlap fra i differenti quadri istopatologici collegabili alle varie sindromi vasculitiche,
anche se in alcuni di essi si può osservare una prevalenza di specifici infiltrati cellulari: linfociti, monociti,
granulociti neutrofili ed eosinofili, e/o cellule giganti di derivazione macrofagica. Altri aspetti riscontrabili
con una certa frequenza a livello della parete vascolare sono la necrosi fibrinoide o il fenomeno
della cosidetta leucocitoclasia (dissoluzione di leucociti con frammentazione nucleare), così come la
formazione di tipiche lesioni granulomatose. La flogosi della parete vascolare facilmente induce fenomeni
trombotici ed ischemici a livello delle diramazioni vascolari nei vari organi coinvolti; quando
invece viene danneggiata la parete di un vaso di medio-grosso calibro, vi può essere un cedimento
strutturale che provoca lesioni aneurismatiche e manifestazioni emorragiche, oppure si generano le
caratteristiche stenosi cinconferenziali
Inovativna uporaba magnezijeva oksida u liječenju "neuralgije celijačnog pleksusa reumatoidnog podrijetla" G. Moscatija 1923.
Get PDFWe presented and discussed one interesting medical prescription by doctor Giuseppe Moscati (1880-1927), who prescribed magnesium oxide (magnesia usta) to a patient with the diagnosis of “neuralgia of the celiac plexus of rheumatic origin”. Besides the traditional use of magnesium as antacid remedy at the time, we raised the hypothesis that magnesium could be administered by Moscati in order to treat the neuralgia itself. Considering the scientific background of Moscati at the school of Filippo Bottazzi (1867-1941), a father of Italian biochemistry, we suggested that the doctor tried to apply the preliminary concepts acquired from electrophysiological studies on magnesium to his clinical practice. Only after decades,
magnesium was recognized a fundamental ion in the energy metabolism and in contributing to maintain the ionic intracellular homeostasis, including for neurons.Prikazali smo i razmotrili zanimljivu medicinsku terapiju doktora Giuseppea Moscatija (1880. – 1927.) koji je propisao magnezijev oksid (magnesia usta) pacijentu s dijagnozom “neuralgije celijačnog pleksusa reumatoidnog podrijetla”. Osim tradicionalne upotrebe magnezija kao antacidnog lijeka u to doba, pretpostavili smo da je Moscati propisao magnezij kako bi liječio samu neuralgiju. S obzirom na znanstvenu pozadinu Moscatija u školi Filippa Bottazzija (1867. – 1941.), oca talijanske biokemije, sugerirali smo da je liječnik pokušao primijeniti preliminarne koncepte dobivene iz elektrofizioloških istraživanja na magneziju u svoju kliničku praksu. Tek nakon nekoliko desetljeća magnezij je prepoznat kao temeljni ion u energetskom metabolizmu koji pridonosi održavanju ionske intracelularne homeostaze, uključujući i neurona
L-Arginine in pregnant scleroderma patients
No full textSystemic sclerosis (SSc) pregnant women show a high frequency of premature births and occurrence of renal crisis. Some evidences showed the role of L-arginine in the prevention and treatment of preeclampsia. Here, we report our experience on the effect of L-arginine treatment in four consecutive SSc pregnant women. Two patients, who have planned the pregnancy, were treated with oral L-arginine; both delivered healthy babies without any prenatal complications. The other two, with high risk of pregnancy complications because of severe lung involvement and type 1 diabetes, respectively, underwent i.v. L-arginine: patient 3 had a premature delivery of a 2-kg healthy baby, while patient 4 developed preeclampsia and, at the 28th week, delivered a 1,050-g girl. The neonate had severe respiratory distress syndrome complicated by severe infection and died at day 28. Although limited, our pilot study suggests that L-arginine may be a useful therapeutic agent in pregnant SSc women
Treatment of severe scleroderma skin ulcers with recombinant human erythropoietin
No full textSystemic sclerosis (SSc) is frequently complicated by skin ulcers, often unresponsive to traditional treatments. A preliminary evaluation of the effects of recombinant human erythropoietin (rHuEPO) was carried out in 14 patients with SSc with nonhealing, severe cutaneous ulcers. Patients received rHuEPO subcutaneously at a dosage of 150 IU/kg 3 times weekly for 2 weeks, twice weekly for the next 2 weeks, and then once weekly for 1 month. At follow-up 3-6 months from the beginning of the treatment, six patients showed complete resolution of the skin ulcers, while a significant reduction (> 60%) in lesional areas was obtained in the other eight patients (mean +/- SD ulcer area reduced from 27.6 +/- 28 to 5.3 +/- 7.8 cm(2); P<0.005). Moreover, patients' quality of life significantly improved (pain, as measured on visual analogue scale reduced from 96 +/- 5 to 46 +/- 17 points; P=0.0001; disability as measured by the Health Assessment Questionnaire-Disability Index reduced from 1.6 +/- 0.5 to 0.9 +/- 0.4 points; P=0.0001). The rHuEPO may represent a novel treatment of nonhealing scleroderma skin ulcers, suggesting some important aetiopathological implications
Vasculiti sistemiche
No full textIl termine vasculiti sistemiche (VS) si riferisce ad ungruppo di condizioni morbose caratterizzate da flogosie necrosi della parete dei vasi sanguigni e da una marcataeterogeneità dal punto di vista eziopatogenetico,istopatologico e clinico-prognostico. Il processo patogeneticoalla base delle differenti VS può coinvolgere oltre allearterie di vario calibro, anche i vasi venosi ed i piccoli vasi(arteriole, capillari e venule). La vasculite è la risultante diun processo immuno-mediato a cui partecipano più costituenticellulari e fattori circolanti quali autoanticorpi, immunocomplessi,complemento ed altri mediatori della flogosi.Vi è un certo overlap (sovrapposizione) fra i differentiquadri istopatologici collegabili alle varie sindromi vasculitiche,anche se in alcuni di essi si può osservare unaprevalenza di specifici infiltrati cellulari: linfociti, monociti,granulociti neutrofili ed eosinofili, e/o cellule giganti diderivazione macrofagica. Altri aspetti riscontrabili con unacerta frequenza a livello della parete vascolare sono la necrosifibrinoide o il fenomeno della cosidetta leucocitoclasia(dissoluzione di leucociti con frammentazione nucleare),così come la formazione di tipiche lesioni granulomatose.Il danno endoteliale quasi sempre associato al processovasculitico può portare a fenomeni trombotici ed ischemicia livello dei vari organi coinvolti, mentre il danno dellaparete vascolare più o meno esteso è spesso responsabiledi lesioni aneurismatiche, a loro volta causa di manifestazioniemorragiche, o stenotiche
Impegno cardiaco nella sclerosi sistemica
No full textL'impegno cardiaco in corso di sclerosi sistemia consta di uno spettro variabile di manifestazioni cliniche conseguenti ad alterazioni di natura microangiopatica, elettrica e/o emodinamica. In danno ischemico e/o flogistico autoimmune del miocardio producono, in ultima analisi, il quadro istopatologico caratteristico della 'necrosi a bande' con deposizione di tessuto fibroso, responsabile della riduzione di elasticità e della capacità contrattile muscolare. ...
Oxycodone in the Long-Term Treatment of Chronic Pain Related to Scleroderma Skin Ulcers.
No full textAbstract Objective. To demonstrate the efficacy and safety of long-term therapy with oxycodone in severe pain of scleroderma skin ulcers. Design. Open study. Setting and Patients. Twenty-nine consecutive patients, referred to our Rheumatology Unit during 2006, affected by systemic sclerosis complicated by painful long-standing skin ulcers entered in the study. In all cases, pain was classified as severe according to World Health Organization guidelines, and oxycodone chloridrate (Oxycontin(R); Mundipharma Pharmaceuticals, Milan, Italy) was administrated at the dosage of 10-20 mg twice daily for a mean period of 7.9 +/- 3.2 standard deviation months. Outcome Measures. To evaluate the efficacy and safety of opioid therapy, the following parameters were recorded at standard time intervals: visual analog scale (VAS) pain, Pittsburgh sleep quality index (PSQI), hours of sleep per night, Health Assessment Questionnaire-Disability index, analgesics use (rescue therapy), side effects, vital signs, routine laboratory assessment. Results. After 1 month of therapy, all patients experienced relief of pain (VAS decreased from 93.8 +/- 8.72 to 56.7 +/- 10.4, P < 0.0001), and better quality of sleep (total hours of sleep increased from 3.68 +/- 1.28 to 5.27 +/- 0.75, P < 0.0001; PSQI decreased from 9.72 +/- 3.95 to 3.37 +/- 1.04, P < 0.0001). These parameters further improved after 3 months of therapy and remained stable during the follow-up; moreover, an increase of daily dosage of oxycodone was never required. The observed side effects were always transient and mild; only constipation, when present, was persistent. Conclusion. Oxycodone showed to be effective and safe in the treatment of pain due to severe scleroderma skin ulcers; contemporarily, it markedly improved the patient's compliance to local wound care procedures
Severe alopecia complicating systemic sclerosis
No full textAims: To describe a case of systemic sclerosis (SSc) associated with severe alopecia areata (AA) responsive to topical and systemic treatments, including vasoactive and immunosuppressive drugs (mycophenolate mofetil).
Presentation of the Case: A 56 year old woman, affected by SSc as from 5 years back, developed a rapid hair loss that progressively involved a large area of the scalp. AA was diagnosed, after the exclusion of an overlapping systemic lupus erythematosus or fungal infection. Treatment with topical steroids and minoxidil, plus mycophenolate mofetil that was introduced for interstitial lung disease, led to progressive improvement of alopecia up to a complete resolution
within 4 months.
Discussion: This is an interesting observation of SSc complicated by severe AA, which is often observed in patients affected by various autoimmune disorders. A possible common pathogenesis of AA and SSc is also discussed
- …
