| Abstract | Improved understanding of the pathophysiology of systemic sclerosis (SSc) opens new therapeutic avenues in its treatment. The efficacy of disease-modifying agents remains limited however, and none has yet demonstrated its ability to improve survival in a prospective randomized trial. RESULTS: of traditional antifibrotic agents such as colchicine and D-penicillamine are disappointing. Cyclophosphamide (CYC) seems to be beneficial in interstitial lung disease associated with SSc. Organ-specific therapies may produce dramatic benefits. Examples include angiotensin-converting enzyme inhibitors for renal failure and epoprostenol for primary pulmonary hypertension. Several new therapeutic approaches are currently under evaluation, including high-dose CYC followed by peripheral stem cell transplantation, vasodilators, and antiinflammatory and antifibrotic agents. Physical therapy and rehabilitation may help to treat disability and loss of function in SSc patients. |
| Authors | Luc Mouthon, Alice Berezné, Serge Poiraudeau, Loïc Guillevin
(Affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Faculté de Médecine. luc.mouthon at cch.aphp.fr)
|
| Journal | Presse médicale (Paris, France : 1983)
(Presse Med)
Vol. 35
Issue 12 Pt 2
Pg. 1975-82
(Dec 2006)
ISSN: 0755-4982 France |
| Vernacular Title | Prise en charge thérapeutique de la sclérodermie systémique. |
| PMID | 17159724
(Publication Type: Comparative Study, English Abstract, Journal Article, Review)
|
| Chemical References |
- Angiotensin-Converting Enzyme Inhibitors
- Anti-Inflammatory Agents
- Anti-Inflammatory Agents, Non-Steroidal
- Antihypertensive Agents
- Immunosuppressive Agents
- Vasodilator Agents
- Epoprostenol
- Cyclophosphamide
- Penicillamine
- Cysteine
- bucillamine
- Interferons
|
| Topics |
- Angiotensin-Converting Enzyme Inhibitors
(administration & dosage, therapeutic use)
- Anti-Inflammatory Agents
(therapeutic use)
- Anti-Inflammatory Agents, Non-Steroidal
(administration & dosage, therapeutic use)
- Antihypertensive Agents
(administration & dosage, therapeutic use)
- Cyclophosphamide
(administration & dosage, therapeutic use)
- Cysteine
(administration & dosage, analogs & derivatives, therapeutic use)
- Epoprostenol
(administration & dosage, therapeutic use)
- Humans
- Hypertension, Pulmonary
(drug therapy, etiology)
- Immunosuppressive Agents
(administration & dosage, therapeutic use)
- Interferons
(administration & dosage, therapeutic use)
- Kidney Failure, Acute
(drug therapy, etiology)
- Lung Diseases, Interstitial
(drug therapy, etiology)
- Orthotic Devices
- Penicillamine
(administration & dosage, therapeutic use)
- Peripheral Blood Stem Cell Transplantation
- Physical Therapy Modalities
- Prospective Studies
- Randomized Controlled Trials as Topic
- Retrospective Studies
- Scleroderma, Systemic
(complications, drug therapy, mortality, physiopathology, rehabilitation, therapy)
- Vasodilator Agents
(therapeutic use)
|