Atteinte digestive de la sclérodermie systémique

Gastrointestinal tract involvement in systemic sclerosis concerns more than 90% of patients but is of heterogeneous clinical expression. It can involve the entire intestinal tract and be responsible for multifactorial malnutrition, which is frequent in this disease. It is a major source of deterioration in the quality of life and can even be life-threatening. Management is complex and multidisciplinary, ranging from simple hygienic and dietary measures, to specialized endoscopic or surgical interventional procedures, also including medical treatments, particularly proton pump inhibitors and prokinetics, with potential side effects. Ongoing research for new diagnostic and therapeutic tools promises to improve the management and prognosis of these patients.     

Potentialités et intérêt du tissu adipeux dans la sclérodermie

Systemic sclerosis is a disorder involving the connective tissue, arterioles and microvessels. It is characterized by skin and visceral fibrosis and ischemic phenomena. Currently, therapy is limited and no antifibrotic treatment has proven its efficacy. Beyond some severe organ lesions (pulmonary arterial hypertension, pulmonary fibrosis, scleroderma renal crisis), which only concern a minority of patients, the skin sclerosis of hands and face and the vasculopathy lead to physical and psychological disability in most patients. Thus, functional improvement of hand motion and face represents a priority for patient therapy. Due to its easy obtention by fat lipopaspirate and adipocytes survival, re injection of adipose tissue is a common therapy used in plastic surgery for its voluming effect. Identification and characterization of the adipose tissue-derived stroma vascular fraction, mainly including mesenchymal stem cells, have revolutionized the science showing that adipose tissue is a valuable source of multipotent stem cells, able to migrate to site of injury and to differentiate according to the receiver tissue's needs. Due to easy harvest by liposuction, its abundance in mesenchymal cells far higher that the bone marrow, and stroma vascular fraction's ability to differentiate and secrete growth angiogenic and antiapoptotic factors, the use of adipose tissue is becoming more attractive in regenerative medicine. We here present the interest of adipose tissue use in the treatment of the hands and face in scleroderma.     

Œsophagite par reflux au cours de la sclérodermie systémique: prévalence et facteurs de risque

Aims

To evaluate the prevalence of reflux oesophagitis (RE) in systemic sclerosis (SS), define its severity and analyze the risk factors.

Patients and methods

The study involved 173 consecutive cases of SS recruited prospectively over a period of 18 years (between April 1988 and June 2006, on 1980 ACR criteria). The mean age is 40.6 ± 13.4 years, the sex-ratio 0.14 and the average duration 6.7 ± 7.6 years. According to LeRoy et al. classification, 141 cases involved a located cutaneous form (81.5%) and 32 cases a diffuse form (18.5%). All the patients underwent a questionnaire concerning the presence and gradation of symptoms of reflux and/or dysphagia, an endoscopy and a standard oesophageal manometry. Twenty-four hour pH Monitoring was carried out on 89 patients.

Results

RE was found in 62 patients (35.8%), being mild or moderate (A and B LA) in 41 cases (23.7%) and severe (C and D) or complicated in 21 cases (12.1%). Presence of RE is not influenced by age, sex, duration or cutaneous extension of the disease. On the other hand, presence of oesophageal symptoms is a good indicator of the presence of mucous lesions. A pathological acid GER is almost constant in cases of RE in both upright and supine positions. GER index is higher in presence of RE than in its absence. The presence of the latter is statistically dependent on both lower oesophageal sphincter (LES) incompetence and oesophageal motor disorders. Moreover, the LES pressure and the distal oesophageal contractile amplitude wave are significantly lower in presence of RE than in its absence.

Conclusions

Reflux oesophagitis is observed in over 30% of scleroderma. Its presence is not related to the age, sex, duration or extension of the disease. It is especially linked to the presence of GER and severe oesophageal motor disorders.