Marie-France Hellot

Esophageal involvement and pulmonary manifestations in systemic sclerosis.

OBJECTIVE To assess whether esophageal manometric motor disturbances are associated with abnormalities consistent with interstitial lung disease (ILD) on both pulmonary function tests (PFT) and high resolution computerized tomography (HRCT) scans in patients with systemic sclerosis (SSc), during initial evaluation and at 2 years followup. METHODS Esophageal manometry, PFT, and HRCT scans were performed in 43 consecutive SSc patients. PFT and HRCT scan parameters were compared between patients with severe esophageal motor dysfunction (i.e., aperistalsis and decreased low esophageal sphincter pressure), patients with moderate esophageal motor dysfunction (hypoperistalsis), and patients without esophageal motor dysfunction on manometry. RESULTS During initial evaluation of SSc, patients with severe esophageal motor impairment, compared with those with moderate and without esophageal dysmotility, exhibited significantly decreased median values of diffusing capacity for carbon monoxide (DLco) (68% vs. 94% vs. 104%) and exhibited higher prevalence of evidence for ILD on HRCT scan (57% vs. 27% vs. 18%). At 2 years followup of SSc, patients with severe esophageal motor disturbances, compared with those without, had faster deterioration of DLco median values (-16% vs. +1%) and higher frequency of ILD on HRCT scan (70% vs. 25%). CONCLUSION Our series underscores a correlation between the degree of esophageal manometric motor disturbances and evidence for ILD on PFT and HRCT scan in SSc patients, suggesting that gastroesophageal reflux (GER) may be one of the contributing factors of ILD in SSc. Our findings further indicate that patients with severe esophageal impairment may require closer followup of lung parameters. In turn, it suggests that aggressive therapy of GER should be initiated in these SSc patients, as it may result in decreased deterioration of pulmonary function.

Influence of age on characteristics of polymyositis and dermatomyositis in adults.

In a retrospective study of 79 consecutive patients, we evaluated characteristics of polymyositis (PM) and dermatomyositis (DM) and compared clinical presentation, biochemical findings, histologic changes, evolution, complications, and mortality rate of elderly patients (aged > or = 65 yr) and younger patients (aged < or = 64 yr) at the onset of PM/DM. We found a high prevalence of PM/DM in elderly patients: 23 patients (29%) were aged 65 years or over. We also found that esophageal involvement (34.8% versus 16.1%, respectively) and bacterial pneumonia related to both ventilatory insufficiency and esophageal impairment (21.7% versus 5.4%, respectively) were more common in elderly patients compared with younger patients, resulting in increased morbidity and mortality rates. Moreover, malignancy frequency was higher in elderly patients compared with younger patients (47.8% versus 9.1%, respectively, p = 0.0001), particularly patients with DM (10/11). Fifty percent of malignancies were colon malignancies in elderly patients. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and ferritin levels were also higher in the elderly patient group compared with the younger group, and the presence of serum hypoprotidemia, hypoalbuminemia, and anemia was more frequent. Finally, PM/DM complete remission was less frequent (13.6% versus 41.1%) and the mortality rate (47.8% versus 7.3%) was higher in elderly patients than in younger patients. The main causes of death in elderly patients were bacterial pneumonia, due to ventilatory insufficiency and esophageal impairment, and malignancies. Our findings therefore indicate that PM/DM-related esophageal and lung involvement should be systematically searched for in elderly patients. Esophageal manometry and pulmonary function tests should become an integral part of initial evaluation in elderly patients for early detection of impairment. Moreover, as we observed a marked overrepresentation of colon cancer in elderly patients with DM, we suggest that the search for malignancies in elderly patients with DM should include lower gastrointestinal tract investigations.

Gastric involvement in systemic sclerosis: a prospective study

OBJECTIVES:This study aims to assess the prevalence of gastric electrical activity dysfunction with cutaneous electrogastrography (EGG), disturbances of gastric emptying function using radiopaque pellets, and gastric endoscopic abnormalities in patients with systemic sclerosis (SSc). We also investigate for an association between EGG and gastric-emptying data with clinical manifestations and esophageal motor disturbances.METHODS:Fasting and postprandial gastric electrical activity was studied in 22 consecutive patients with SSc (17 with and 5 without clinical gastric manifestations) and 22 age- and sex-matched healthy subjects. Gastric emptying of radiopaque pellets and gastroscopy were also performed in SSc patients.RESULTS:The prevalence of EGG disturbances was as high as 81.82% in SSc patients. SSc patients exhibited, compared with controls, higher median percentage of dominant frequency in bradygastria during the fasting period and lower median values for postprandial electrical power and postprandial to fasting ratio for electrical power. Gastric emptying of radiopaque pellets was delayed in 11 SSc patients, and gastroscopy demonstrated “watermelon stomach” in 3 SSc patients. No correlation was found between the severity of gastric impairment and clinical presentation, SSc duration and subsets, and esophageal manometric impairment.CONCLUSIONS:Our study underlines the high frequency of gastric dysfunction in SSc patients. It suggests the usefulness of EGG in SSc in noninvasively detecting disorders of gastric electrical activity at an early stage and symptomatic patients with gastroparesis (because there was a correlation between values of postprandial to fasting ratio for electrical power of < 1 on EGG and delayed gastric emptying). Gastric-emptying evaluation of radiopaque pellets may further be a helpful method to depict symptomatic SSc patients with gastroparesis. Finally, our series confirms that watermelon stomach diagnosis should be excluded in SSc patients presenting with gastrointestinal hemorrhage or with anemia related to iron deficiency.

Neurological disorders in patients with bullous pemphigoid.

Background: Unexpected cases of bullous pemphigoid (BP) have been reported in adult patients with various neurological disorders suggesting a possible relationship between these diseases. Objectives: (1) To determine the prevalence and types of neurological disorders in patients with BP, (2) to assess patients’ functional impairment, and (3) to compare the clinical and biological findings as well as prognosis of BP patients presenting with or without neurological disorders. Methods: BP patients with neurological disorders were selected in a series of 341 consecutive BP patients treated in 20 French Dermatology Departments. Functional impairment was prospectively assessed using the Karnofsky score which is a measure of patients’ general condition. Results: At least one neurological disorder was present in 123 of the 341 BP patients (36%). They primarily consisted of dementia (n = 68; 20%; 95% CI: 16–25%), cerebral stroke (n = 52; 15%; 95% CI: 4–19%), and/or Parkinson’s disease or parkinsonism (n = 32; 9%; 95% CI: 7–13%). BP patients with neurological disease were older than patients without neurological disease (83.8 ± 7.5 years vs. 79.3 ± 10.3 years, p < 10–4). They also had a lower Karnofsky score (47 ± 19% vs. 74 ± 20%, p < 10–4). One-year overall survival rates of the two groups were 50.8% (95% CI: 41.8–59.7) and 78.7% (95% CI: 73.0–84.2), respectively (p < 10–4). In contrast, the number of bullae and main biological features at baseline were not different between the two groups of patients. Conclusion: This study demonstrated a high frequency of neurological disorders, particularly dementia, in BP patients. Most of these patients had a severe functional impairment and a poor prognosis.

Oesophageal mucosal involvement in patients with systemic sclerosis receiving proton pump inhibitor therapy

Aim  To assess the prevalence of oesophagitis, Barretts oesophagus (BE) and other oesophageal mucosal abnormalities in patients with systemic sclerosis (SSc) without prior selection on digestive clinical presentation. We also investigated the association between oesophageal endoscopic and manometric data with clinical manifestations of SSc.

Manometry of the upper intestinal tract in patients with systemic sclerosis: A prospective study

OBJECTIVE To assess both the prevalence and the characteristics of motor disorders of the small bowel in patients with systemic sclerosis (SSc) and to investigate for an association between clinical manifestations in the upper intestinal tract, capillaroscopic features, esophageal motor impairment, and manometric evidence of motor disturbances. METHODS Fasting and postprandial motor activity of the upper intestinal tract was studied in 17 consecutive patients with SSc (6 with and 11 without clinical manifestations of small bowel involvement) and 17 age- and sex-matched healthy control subjects. RESULTS The prevalence of manometric evidence of intestinal involvement was as high as 88% in the SSc patients; normal motor activity was present in only 2 patients. The median values for duodenal and jejunal interdigestive phase III migrating motor complex duration, amplitude, and velocity and the postprandial motility index were therefore lower in SSc patients compared with controls. Our manometric findings indicated that there are both neuropathic and myopathic stages of upper intestinal tract dysfunction in SSc. Furthermore, no association could be found between the severity of the intestinal manometric abnormalities and clinical presentation, SSc subsets, disease score, capillaroscopic findings, or esophageal manometric impairment. CONCLUSION We suggest that manometry of the upper intestinal tract may be useful in SSc patients with clinical manifestations in the small bowel (i.e., malabsorption syndrome or pseudoobstruction) in that it can be used to accurately evaluate both the nature and the severity of motor disturbances. Furthermore, this procedure can be used to assist in the selection of patients who may require octreotide therapy.

Noninvasive mechanical ventilation in clinical practice: A 2-year experience in a medical intensive care unit

ObjectiveTo evaluate the feasibility and outcome results of noninvasive mechanical ventilation (NIV) in daily clinical practice outside any prospective protocol-driven trial. DesignAn observational retrospective cohort study. SettingA 22-bed medical intensive care unit in a university hospital. PatientsA consecutive cohort of 124 patients who underwent 143 NIV trials, regardless of the indication, over two consecutive years (1997–1998). InterventionsNone. ResultsA total of 604 acute respiratory failure patients underwent mechanical ventilation, and 143 NIVs were performed in 124 patients. The overall prevalence of NIV use was 143 of 604 patients (24%) in three groups: hypoxemic acute respiratory failure (29.5%), hypercapnic acute respiratory failure (41%), and weaning/postextubation (29.5%). Intubation was avoided in 92 of 143 of the NIVs performed (64%), 19 (13%) after changing the initial NIV mode (i.e., a success rate of 62%, 51%, and 86% in the three groups, respectively). A total of 35 of 51 intubated patients (69%) required intubation during the first 24 hrs of NIV. Intensive care unit stay was 12 ± 10 days for the overall population, and mortality, when NIV failed, was 13 of 124 patients (10.5%). Arterial pH (p = .0527) and the Pao2/Fio2 ratio (p = .0482) after 1 hr were the only independent predictive factors for NIV failure by multivariate analysis. ConclusionsThis study confirms the results of controlled trials and demonstrates the feasibility and efficacy of NIV applied in daily clinical practice. These results suggest that NIV should be considered as a first-line ventilatory treatment in various etiologies of acute respiratory failure and as a promising weaning technique and postextubation ventilatory support. However, NIV should certainly be performed by a motivated and sufficiently trained care team.

Interface strategy during noninvasive positive pressure ventilation for hypercapnic acute respiratory failure

Objective:To assess the influence of initial mask choice on the clinical effectiveness and tolerance of noninvasive positive pressure ventilation (NIPPV) in the management of hypercapnic acute respiratory failure. Design:A prospective randomized controlled clinical study. Setting:A medical intensive care unit at a university hospital. Intervention:Randomization between two NIPPV interfaces. Patients:Initial mask choice was randomized between two standard masks: face (NIPPVf group) and nasal (NIPPVn group). The main end point was mask failure (i.e., mask change and/or intubation). Secondary end points were tolerance of NIPPV, change in respiratory parameters during the first 3 days, and patient outcome. Results were analyzed on an intent to treat basis. A per protocol analysis was also conducted. Main Results:Ninety patients with underlying chronic lung disease were included, 46 in the NIPPVf group and 44 in the NIPPVn group. The overall success rate of NIPPV was 83%. Mask failure occurred significantly more often in the NIPPVn group (32/44 vs. 9/46; p < 0.0001), mainly because of the need for mask change (32/44 vs. 0/46; p < 0.0001) because of the occurrence of major buccal air-leaks in 94% of cases. Improvement in respiratory parameters was similar in the two groups. Whereas air-leaks were more frequent in the NIPPVn group (p < 0.05), respiratory comfort was assessed as lower and complications more frequent by the staff in the NIPPVf group from day 2 (p < 0.05). Conclusions:A face mask should be the first-line strategy in the initial management of hypercapnic acute respiratory failure with NIPPV. However, if NIPPV has to be prolonged, switching to a nasal mask may improve comfort by reducing face mask complications.

Prior chemoradiotherapy is associated with a higher life-threatening complication rate after palliative insertion of metal stents in patients with oesophageal cancer

Self‐expanding metal stents are used routinely to palliate dysphagia due to oesophageal cancer.

Induction cisplatin–irinotecan followed by concurrent cisplatin–irinotecan and radiotherapy without surgery in oesophageal cancer: multicenter phase II FFCD trial

A recent phase I study showed that weekly cisplatin, irinotecan and concurrent radiotherapy can be administered with moderate toxicity in patients with oesophageal cancer. Patients with no prior treatment and oesophageal cancer stage I to III, performance status <3, caloric intake >1500 kcal day−1 were included. Chemotherapy, with cisplatin 30 mg m−2 and irinotecan 60 mg m−2, was administered at days 1, 8, 22, 29, and concurrently with radiotherapy at days 43, 50, 64 and 71. Radiotherapy was delivered with 50 or 50.4 Gy in 25 fractions/5 weeks. Forty-three patients were included, 10 stage I, 19 stage II and 14 stage III. Mean age was 59.2 years (range 44–79). A total of 30 out of 43 (69.8%) patients underwent all planned treatment. During induction chemotherapy, 14 severe toxicities of grade 3 or 4 in 10 patients (23.3%) were reported with 57.1% due to haematoxicity. During chemoradiotherapy, 31 severe toxicities of grade 3 or 4 with 64.5% due to haematotoxicity were reported in 18 patients. One toxic death occurred (diarrhoea grade 4). The complete clinical response rate was 58.1% (95% CI: 43.4–72.8%). Overall survival rate at 1 and 2 years was 62.8%, (95% CI, 58.3–77.3%) and 27.9% (95% CI, 13.4–41.3%), respectively. In conclusion, cisplatin–irinotecan–radiotherapy is an active and well-tolerated regimen feasible in out-patients.