Analysis of the dental care queries in the "Mouth-Nose" discussion forum of the French association of patients with Gougerot-Sjögren's syndromes and dryness.

BACKGROUND: Primary Sjögren Syndrome is a rare autoimmune systemic disease characterized by impaired secretory functions of the exocrine gland. One of the main clinical features is dry mouth and subsequent oral diseases, which are also found in patients with Sicca. This leads to a marked deterioration in the quality of life and the patient's search for information and solutions. Many patients turn to patients' associations that offer moments of sharing to their members, especially through online discussion forums. Today, these forums represent quality material for a sociological or biomedical analysis of patients' concerns, as close as possible to their daily lives. Our objective is to analyze the concerns of patients with SS or Sicca regarding their dry mouth especially dental care. METHODS: In this cross-sectional observation study, a quantitative analysis of the Mouth-Nose online forum discussion of the French Association of Patients with Gougerot-Sjögren's Syndromes and Dryness have been performed. After reading and re-reading, initial request themes, topics, and subtopics were established and coding was performed. Then, the 885 threads were classified depending the initial request, pragma-linguistic indices and the main topic discussed in the thread. After identifying the threads dealing with dental care, we looked at which types of care were most discussed and classified the discussions according to whether or not the patient was satisfied with their care at the dentist. RESULTS: The majority of the initial requests are posts for experiences sharing and/or advice. The topic of "dental care" is one of the main concerns of the forum users. Among the threads that concern dental care, requests to share experience with implants are in the majority. Finally, the majority of the posts on dental care relate to care in private dental practice, deals with dental implants and prevention and resulted mainly in patient satisfaction. CONCLUSIONS: Analysis of the forum reveals importance of patient concerns about prevention, and care costs due to implant treatment, which add to disease burden. Most of messages relate favorable experiences with their dentists, which is in line with the approach of sharing experiences and support characteristic of a forum.

Weight of salivary gland ultrasonography compared to other items of the 2016 ACR/EULAR classification criteria for Primary Sjögren's syndrome.

OBJECTIVE: Major salivary gland ultrasonography (SGUS) is widely used for the diagnosis of primary Sjögren's syndrome (pSS). Our objective was to assess the contribution of SGUS compared to other items of the 2016 ACR/EULAR pSS classification criteria, based on expert opinion. METHODS: A secure web-based relational database was used by 24 experts from 14 countries to assess 512 realistic vignettes developed from data of patients with suspected pSS. Each vignette provided classification criteria items and information on history, clinical symptoms and SGUS findings. Each expert assessed 64 vignettes, and each vignette was assessed by 3 experts. A diagnosis of pSS was defined according to at least 2 of 3 experts. Validation was performed in the independent French DiapSS cohort of patients with suspected pSS. RESULTS: A criteria-based pSS diagnosis and SGUS findings were independently associated with an expert diagnosis of pSS (P < 0.001). The derived diagnostic weights of individual items in the 2016 ACR/EULAR criteria including SGUS were as follows: anti-SSA, 3; focus score ≥ 1, 3; SGUS score ≥ 2, 1; positive Schirmer's test, 1; dry mouth, 1; and salivary flow rate < 0.1 mL/min, 1. The corrected C statistic area under the curve for the new weighted score was 0.96. Adding SGUS improves the sensitivity from 90.2 % to 95.6% with a quite similar specificity 84.1% versus 82.6%. Results were similar in the DiapSS cohort: adding SGUS improves the sensitivity from 87% to 93%. CONCLUSION: SGUS had similar weight compared to minor items, and its addition improves the performance of the 2016 ACR/EULAR classification criteria.

Pregnancy and newborn outcomes after exposure to bisphosphonates: a case-control study.

We analyzed women and newborn outcome after maternal exposure to BPs. BPs have no teratogenic effect on the 36 analyzed pregnancies compared to unexposed controls matched on women underlying diseases (either systemic disease, either "bone" disease) but some outcome differed: neonatal complications rate in systemic diseases and live birth rate in bone diseases). INTRODUCTION: The effect of bisphosphonates (BPs) during pregnancy remains unclear. We aimed to study pregnancy outcomes in women exposed to BPs during pregnancy. METHODS: Data for cases and controls were from the French Reference Centre of Teratogenic Agents. Cases were women who received BPs in the 6 weeks before or during a pregnancy and had systemic or bone diseases. We included two respectively matched control groups: women with systemic diseases not exposed to BPs and healthy women not exposed to BPs or any teratogenic agent. Four controls were assigned to each case. RESULTS: Thirty-six women were exposed to BPs including 5 just before pregnancy and 30 during the first trimester; 23 had systemic diseases (systemic lupus erythematosus, n = 5; rheumatoid arthritis, n = 5; other, n = 13) and 13 had bone diseases. Rate of observed congenital malformations did not differ in women with a systemic or a bone disease compared to their respective controls (respectively 2/23 [8.7%] vs 2/92 [2.2%], p = 0.178 and 0/13 [0%] vs 0/52 [0%], p = 1.00). Among women with systemic diseases, non-specific neonatal complications were more frequent for cases (4/16 [25.0%] vs 4/64 [6.3%], p = 0.027). Among women with bone disorders, the live birth rate was lower for cases than healthy controls (8/10 [80%] vs 50/50 [100%], p = 0.025). CONCLUSION: We found no major teratogenic effects of BPs, but rates of neonatal complications were increased for women with systemic diseases, as were spontaneous abortions for women with bone diseases likely linked to the severity of the underlying diseases and concomitant medications.

Efficacy of abatacept in systemic lupus erythematosus: a retrospective analysis of 11 patients with refractory disease.

Objective The objective of this study was to assess the safety and efficacy of abatacept in patients with SLE refractory to conventional treatment in routine clinical practice. Methods This retrospective study included 11 SLE patients treated with abatacept for an active and refractory disease. The primary endpoint was the change in SLE Disease Activity Index (SLEDAI) score at six months. Response was defined as a decrease of SLEDAI ≥4 in a patient continuing abatacept. Results Indications of abatacept treatment were articular ( n=8), renal ( n=1) and cutaneous ( n=1) involvement and autoimmune thrombocytopenia ( n=1). Abatacept was discontinued before six months in two patients, because of adverse event ( n=1) and/or lupus flare ( n=2). The median SLEDAI decreased from 6 (2-20) to 4 (0-20) ( p=0.031). Decrease of SLEDAI ≥4 was observed in 6/11 patients (55%) and response to treatment according to the physician's judgement in 8/11 (73%) patients. Improvement of articular involvement was observed in 7/8 (87.5%) patients. Four adverse events were observed in three patients, but no severe infection occurred. Conclusion This study suggests some efficacy of abatacept in patients with refractory disease in routine clinical practice, particularly in the case of articular manifestations, with an acceptable safety profile. These data support conducting new controlled trials of abatacept in SLE patients.

Upregulation of membrane-bound CD40L on CD4+ T cells in women with primary Sjögren's syndrome.

Epigenetic deregulation of genes encoded on the X chromosome as reported for CD40L in lupus could explain the female predominance of autoimmune diseases. We compared CD40L expression on CD4(+) T cells from primary Sjögren's syndrome (pSS) women and healthy controls and investigated DNA methylation patterns of the promoter and enhancer regions of CD40L. The expression of CD40L on activated CD4(+) T cells was higher in patients with pSS than controls after phorbolmyristate acetate and ionomycin activation (P = 0.02). CD40L mRNA level in CD4(+) T cells did not differ between patients with pSS and controls and was similar in both groups in cultures treated with the demethylating agent 5-azacytidine C. Pyrosequencing analysis revealed no significant differences in methylation profiles between patients and controls. Inducible membrane-bound CD40L on CD4(+) T cells is increased in patients with pSS but was not related to epigenetic deregulation by demethylation patterns of the regulatory regions of CD40L.

Chemosensory dysfunction in primary Sjögren's syndrome: a topical review.

Primary Sjögren's syndrome is an autoimmune exocrinopathy related to lymphocytic infiltration of the exocrine glandular epithelia (such as salivary, lacrimal, nasal, and sebaceous glands or vaginal mucosa) with systemic manifestations of an immuno-inflammatory nature, and not associated with any other systemic disease. It is characterized by severe dryness (Sicca syndrome), particularly in mouth and eyes, with potential strong impact on quality of life and could increase the risk of depression in Sjögren's patient. To date, the impairment of taste and olfactory functions related to Sjögren syndrome remains poorly assessed; so is the trigeminal functions which remain sparsely studied in patients with Sjögren disease. However, other factors can also modify chemosensory functions (olfactory or gustatory sensations and trigeminal nerves), in particular the reduction of the masticatory coefficient or halitosis, due to oral saliva flow decrease, and poor dental condition, which are often present in Sjögren patients. Of the 12 articles evaluated after a 22-year literature search of this review, chemosensory disorders (including taste, smell, and trigeminal impairments) are described and evaluated in pSS patients, with mainly poorer performance compared to healthy controls. Diagnostic and therapeutic (including rehabilitation) approaches of chemosensory disorders in pSS are discussed in this review. Clinician should be more attentive to taste as well as olfacto-trigeminal disorders in primary Sjögren's disease, if possible at the earlier stage, in order to take the best care of Sjögren's patients. This review also highlights some lack in knowledge on pSS chemosensory disorders that should provide new research perspectives. Key Points •Chemosensory functions (including taste, smell, and trigeminal functions) are altered in patients with primary Sjögren's syndrome (pSS) due to dryness of the mouth and the nose. •The trigeminal nerve which interacts with olfactory and gustatory nerves contributes to olfactory and taste perception but remains little studied to date. •Chemosensory function should be considered in the daily clinical assessment of patients with pSS. •Chemosensory function treatment is not standardized yet, however symptomatic treatment of Sjögren syndrome-associated dryness transiently would improve taste and smell, and olfactory or gustatory rehabilitation in pSS patients would be useful.

Measure of function in rheumatoid arthritis: individualised or classical scales?

BACKGROUND: The Health Assessment Questionnaire Disability Index (HAQ-DI) is the most widely used measure of function in rheumatoid arthritis (RA). OBJECTIVE: To evaluate individualised forms of the HAQ-DI and thus enhance the incorporation of patients' views in outcome assessment. PATIENTS AND METHODS: HAQ-DI data were prospectively obtained from 370 outpatients with RA treated with leflunomide over a 6-month period. At baseline and final visits, patients had to rate the importance they attached to each activity addressed by the 20 HAQ-DI items, and to select the five activities they considered the most important. Different individualised scales were evaluated: scales preserving all domains, in which the score for each item is multiplied by or added to its importance; and scales involving, for each patient, only the five most important items. The psychometric properties of these scales were compared with those of the HAQ-DI. RESULTS: For each HAQ-DI item, severity and importance scores were weakly correlated. Scores for all individualised scales were highly correlated with the HAQ-DI score (r(s)>0.75). All scales had a good internal consistency (Cronbach's alpha 0.87-0.88). Compared with the HAQ-DI, individualised scales did not have better sensitivity to change (standardised response mean 0.64-0.69 vs 0.74). CONCLUSION: Individualised scales have similar properties to the HAQ-DI. However, individualised questionnaires measuring importance gave complementary information to the measure of disability. Individualisation is probably not needed for group assessment in all randomised controlled trials but, the use of individualised questionnaires may be clinically relevant for individual patients with RA.

High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients.

OBJECTIVE: To determine the frequency and risk factors of venous thromboembolic events (VTE) in Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and, the so far unstudied, Churg-Strauss syndrome (CSS) and polyarteritis nodosa (PAN). METHODS: Retrospective, systematic analysis and comparisons were made between the characteristics of patients in the VTE group and non-VTE group. 1130 patients with WG, MPA, CSS or PAN were identified from the French Vasculitis Study Group cohort. RESULTS: During a mean follow-up of 58.4 (45.8) months, 83 VTE occurred in 74 (6.5%) patients, with a median vasculitis-VTE diagnosis interval of 5.8 months (-3 to +156). VTE occurred in seven of 285 (2.5%) patients with PAN, 19 of 232 (8.2%) with CSS, 30 of 377 (8%) with WG and 18 of 236 (7.6%) with MPA. Multivariate analysis retained age, male sex or previous VTE or stroke with motor deficit as being associated with a higher VTE risk. The adjusted odds ratio (95% confidence interval) for VTE was 2.88 (1.27 to 6.50) for patients with WG, MPA or CSS compared with PAN (p = 0.01). CONCLUSIONS: Our results suggest that, like WG and MPA, patients with CSS are at a greater risk of VTE, than those with PAN. The reasons for this difference remain to be elucidated.

Glucocorticoid sparing effect of tumour necrosis factor alpha inhibitors in rheumatoid arthritis in real life practice.

OBJECTIVE: To determine, in real life practice, the impact of anti-tumour necrosis factor (TNF)-alpha on glucocorticoid (GC) use in rheumatoid arthritis (RA) patients. METHODS: This systematic monocenter retrospective before-after study enrolled all RA patients who started their first anti-TNF-alpha treatment between January 2004 and December 2006 and were followed more than 3 months after anti-TNF-alpha initiation. Paired comparisons were performed to compare GC intake during the year before anti-TNF-alpha initiation and during the first year of treatment; each patient was his/her own control. Comparisons between patients who reduced their oral prednisone intake and those who did not, were also performed to identify variables associated with prednisone decrease. RESULTS: 110 patients were included (90 females/20 males, age: 42.1+/-14.4 years, disease duration: 10.6+/-10.2 years). Etanercept was prescribed in 70 (63.6%) patients, adalimumab in 35 (31.8%) and infliximab in 5 (4.6%). At anti-TNF-alpha initiation, 79 patients (71.8%) were taking oral prednisone (mean dose 7.3+/-2.6 mg/d). Of the 82 prednisone-users (74.5% of patients), 62 (75.6%) had lowered prednisone doses, whereas 12 (14.6%) and 10 (12.2%) patients had stable or increased doses, respectively. Twelve patients (15.2%) discontinued oral prednisone. Overall, a significant decrease of 28% of oral prednisone use was observed. The only factors associated with oral prednisone decrease were higher initial prednisone daily doses (p=0.04) and female sex (p=0.04). CONCLUSION: This study showed a significant GC sparing-effect of anti-TNF-alpha in RA patients in real life practice that was observed for oral, parenteral and intra-articular administration, as early as the first 3 months of treatment.

Individualising the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) function subscale: incorporating patient priorities for improvement to measure functional impairment in hip or knee osteoarthritis.

OBJECTIVE: Recommended outcome measures in osteoarthritis are standardised scales identical for each patient. As patient-specific scales are of increasing interest when considering patient priorities in outcome assessment, this study aims to validate individualised forms of the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) function subscale. PATIENTS AND METHODS: WOMAC function subscale data were prospectively obtained from 1218 outpatients with hip or knee osteoarthritis requiring non-steroidal anti-inflammatory drugs. Patients also rated the importance to remove disability in each activity of the WOMAC function subscale, and selected the five activities they considered the most important to be improved upon. After treatment, patients again completed the WOMAC function subscale. Several individualisation methods were evaluated: methods whereby the score of each item is multiplied by, or added to, its importance, and methods based on the five most important activities (WOMAC top 5). Psychometric properties of individualised scales were compared to those of the WOMAC function subscale. RESULTS: The missing data rate was 11%, 13% and 2% for the WOMAC function, its individualised forms and the WOMAC top 5, respectively. Combining severity and importance of each item did not improve the properties of the scales. The WOMAC top 5 was the most responsive scale (standardised response mean: 0.96 vs 0.80, p<0.001). CONCLUSION: Because of its better responsiveness, ease of use, low missing data rate and ability to highlight patient priorities, the WOMAC top 5 could be an interesting tool in therapeutic evaluation in hip or knee osteoarthritis.

[Entrapment neuropathies involving the ulnar nerve at the wrist (and into the hand) and the peroneal nerve]. / Neuropathies compressives du nerf ulnaire au poignet (et dans la main) et du nerf fibulaire commun. Données récentes.

We report our experience with patients who underwent surgery for entrapment neuropathies involving the ulnar nerve at the wrist and into the hand and the peroneal nerve. For the ulnar nerve, the cause of the lesion was identified in all patients, generally a cyst which had developed in the Guyon canal. The patients usually recovered completely. For the peroneal nerve, there was a wide variety of causes, with mucoid cysts frequently involved. Recovery was often incomplete, because of the very marked initial axonal damage. We emphasized the need for rapid diagnosis and surgical treatment.

[Steal syndrome]. / Syndrome de vol vasculaire.

BACKGROUND: Ischemic steal syndrome is a complication of arteriovenous access creation for hemodialysis, and is little known among dermatologists despite the fact is fairly common and has a cutaneous presentation. Herein we report the case of a man presenting with serious ischemic injuries to the fingers associated with steal syndrome. OBSERVATION: A 49-year-old diabetic man presented with painful acrosyndrome of the left hand, with a necrotic ulcer on the third finger appearing 5 months after the creation of an arteriovenous access. Clinical examination showed marked pain relief after manual compression of the fistula. Arteriography showed an overfunctional fistula, causing decreased distal perfusion pressure, and this was consistent with the diagnosis of steal syndrome. Banding of the anastomosis was performed but was insufficient to spare the finger from amputation. DISCUSSION: The aim of our case-report is to increase awareness among dermatologists of this fairly frequent complication of arteriovenous accesses. We discuss the physiopathology, risk factors, clinical features and therapeutic management of this condition. Since diagnosis of steal syndrome is fairly straightforward, early detection is essential. Only adequate surgical management can avoid gangrenous manifestations leading to more or less widespread amputation.

Rheumatoid Factor and Disease Activity Are Independent Predictors of Lymphoma in Primary Sjögren's Syndrome.

OBJECTIVE: To define parameters predictive of lymphoma development in patients with primary Sjögren's syndrome (SS). METHODS: A multicenter case-control survey was performed to identify predictors of lymphoma. Cases were patients who developed lymphoma after diagnosis of primary SS and were mainly recruited through the Club Rhumatismes et Inflammation network. For each case, 2 controls (matched for disease duration and age) were randomly selected among patients with primary SS and without lymphoma. Cases and controls were compared using univariate analysis and then using multivariate analysis to identify independent predictors of lymphoma. RESULTS: One hundred one patients with primary SS and lymphoma were included. Eighty-seven patients were women (86.1%), and the mean ± SD age at lymphoma diagnosis was 57.4 ± 12.6 years. The most frequent histologic type was B cell non-Hodgkin's lymphoma (NHL) in 99 of 101 patients, with marginal-zone lymphoma in 76 of the 99 patients (76.8%) including 58 (58.6%) with lymphoma of the mucosa-associated lymphoid tissue type. Lymphomas were most frequently located in the salivary glands (43 patients). A specific treatment was initiated at diagnosis in 87 patients with B cell NHL, and 61 patients (61.6%) achieved complete sustained remission after the first line of treatment. In the multivariate analysis, salivary gland enlargement, the presence of rheumatoid factor (RF), low C4, cryoglobulinemia, lymphopenia, and disease activity according to the European League Against Rheumatism Sjögren's Syndrome Disease Activity Index (excluding the lymphoma domain) were found to be predictors of lymphoma. No previous treatment for primary SS was associated with any effect on lymphoma occurrence. CONCLUSION: In addition to previously known factors predictive of lymphoma occurrence, the independent roles of RF and disease activity were demonstrated in this case-control study of primary SS-associated lymphoma. Our findings highlight the roles of chronic antigenic stimulation and disease activity in the development of this severe complication.

ACPA-positive primary Sjögren's syndrome: true primary or rheumatoid arthritis-associated Sjögren's syndrome?

OBJECTIVES: Anticyclic citrullinated protein antibodies (ACPA) are highly specific of rheumatoid arthritis (RA). However, they have also been detected in 5-10% of primary Sjögren's syndrome (pSS). We compared ACPA-positive and negative patients with pSS and assessed the risk of evolution to RA. PATIENTS AND METHODS: ACPA-positive and negative patients with pSS were included in this study. For ACPA-positive patients, clinical and radiological re-evaluation was systematically performed after at least 5 years of follow-up. Diagnosis was reassessed at the end of the follow-up to identify patients that developed RA according to the American College of Rheumatology 1987 classification criteria. RESULTS: At inclusion in the cohort 16 patients with pSS were ACPA positive and 278 were ACPA negative. ACPA-positive patients, had more frequently arthritis (43.7% vs 12.2%; p=0.003) but not arthralgias. They also had more frequent lung involvement (25% vs 8.1%; p=0.05). After median follow-up of 8 (5-10) years, 7/16 (43.8%) patients developed RA including 5 (31.25%) with typical RA erosions. Elevation of acute phase reactants at inclusion was the only parameter associated with progression to erosive RA. CONCLUSIONS: Median term follow-up of ACPA-positive patients with pSS showed that almost half of them developed RA, particularly in the presence of elevation of acute phase reactants. These results support the usefulness of a close radiological monitoring of these patients for early detection of erosive change not to delay initiation of effective treatment. Indeed, number of these patients with ACPA-positive pSS may actually have RA and associated SS.

CXCL13 and CCL11 Serum Levels and Lymphoma and Disease Activity in Primary Sjögren's Syndrome.

OBJECTIVE: Non-Hodgkin's lymphoma (NHL) is a severe complication of primary Sjögren's syndrome (SS). Ectopic germinal centers (GCs) in the salivary glands are predictors of the occurrence of NHL. Given the association between CCL11 and CXCL13 and ectopic GCs, we assessed the link between these chemokines and NHL, as well as the association between these chemokines and disease activity, in patients with primary SS. METHODS: Serum levels of CCL11 and CXCL13 were evaluated by multiplex assay in 385 patients included in the Assessment of Systemic Signs and Evolution of Sjögren's Syndrome (ASSESS) cohort. The association between chemokine levels, B cell biomarkers, and patient subsets was assessed using Spearman's test for continuous data and the nonparametric Mann-Whitney U test for categorical data. Multivariate analyses were performed to identify parameters associated with lymphoma and disease activity. RESULTS: Seventeen patients had a history of lymphoma, and 5 of them had developed NHL during followup. The median serum levels of CCL11 and CXCL13 in the total cohort were 106.48 pg/ml (interquartile range 69.33-149.85) and 108.31 pg/ml (interquartile range 58.88-200.13), respectively. Patients with lymphoma had higher levels of CXCL13 than did patients without lymphoma (P = 0.006) and a trend toward a higher level of CCL11 (P = 0.056). Low C4 and high BAFF levels were associated with NHL on multivariate analysis (P = 0.01 and P = 0.0002, respectively). CCL11 and CXCL13 levels correlated positively with the rheumatoid factor titer, the κ-to-λ free light chain ratio, and the ß2 -microglubulin level. CXCL13 was the only parameter associated with disease activity on multivariate analysis. CONCLUSION: These findings demonstrate a link between CXCL13 and CCL11 and disease activity and lymphoma. This highlights the continuum between chronic B cell activation, disease activity, and lymphomagenesis in patients with primary SS.

European League Against Rheumatism Sjögren's Syndrome Disease Activity Index and European League Against Rheumatism Sjögren's Syndrome Patient-Reported Index: a complete picture of primary Sjögren's syndrome patients.

OBJECTIVE: The European League Against Rheumatism (EULAR) Sjögren's Syndrome (SS) Disease Activity Index (ESSDAI) and the EULAR SS Patient-Reported Index (ESSPRI) were recently developed. We aimed to determine whether patients' symptoms differed between patients with and without systemic involvement and if the disease-specific indices correlated with each other in primary SS. METHODS: Fifteen French centers included 395 primary SS patients in the Assessment of Systemic Signs and Evolution in Sjögren's Syndrome Cohort. At enrollment, physicians completed the ESSDAI, the SS Disease Activity Index (SSDAI), and the Sjögren's Systemic Clinical Activity Index (SCAI), and patients completed the ESSPRI, the Sicca Symptoms Inventory, and the Profile of Fatigue and Discomfort. All scores were compared between patients with and without systemic involvement. Correlations between scores of systemic activity and patients' symptoms were obtained. RESULTS: At enrollment, 120 (30.4%) patients had never experienced systemic complication and 155 (39.2%) patients and 120 (30.4%) patients had, respectively, only past or current systemic manifestations. Past or current systemic patients had higher levels of symptoms, except dryness. The ESSDAI did not correlate with the patient-scored ESSPRI (rho = 0.06, P = 0.30), whereas the SSDAI and the SCAI, which include subjective items, did correlate (rho = 0.28 and 0.25, respectively; P < 0.0001 for both). CONCLUSION: Alterations of common patient-reported outcomes are present in all patients with primary SS, including those with systemic complications. However, patient symptoms and systemic complications are 2 different facets of primary SS. Therefore, the use of both systemic and patients' indices, such as the ESSDAI and ESSPRI, are useful. Since these 2 facets weakly overlap, one should identify which of both components is the main target of the treatment to test, when designing clinical trials in primary SS.

Impact of systemic sclerosis on occupational and professional activity with attention to patients with digital ulcers.

OBJECTIVE: To evaluate the impact of systemic sclerosis (SSc; scleroderma) and digital ulcers (DUs) on daily living and professional activities. METHODS: We prospectively evaluated 189 SSc patients for employment status and disability during meetings of the French SSc patient association (n=86, 45.5%) or during hospitalization (n=103, 54.5%). RESULTS: Seventy-eight (41.2%) patients had diffuse SSc. The mean±SD age was 54±13 years, and the mean±SD disease duration was 9.3±8.4 years at the time of evaluation. Sixty (31.7%) patients had at least one DU. Assessed using the Health Assessment Questionnaire (mean±SD 1.12±0.79 versus 1.39±0.84; P=0.001), the Cochin Hand Function Scale (mean±SD 20.2±18.3 versus 27.8±19.1; P<0.0001), and the Hospital Anxiety Scale (mean±SD 9.9±5 versus 8.5±4.2; P=0.04), global disability, hand disability, and anxiety, respectively, were significantly higher in patients with DUs than in others. Most patients reported a limitation in daily activities related to SSc, as assessed by a daily activity limitation scale (mean±SD 4.4±2.9) and an increased need for help in the home. Patients reported needing mean±SD 4±13.5 hours per month of paid household help related to SSc and mean±SD 1.5±10 hours per month related to DUs, with significant differences between patients with or without DUs (P=0.004). Among the 113 patients in the workforce, 67 (59.3%) were employed, 42 (37.2%) were employed full time, 36 (31.8%) received full disability pension, and 27 (23.9%) were on sick leave, with no difference between patients with or without DUs. CONCLUSION: SSc has a significant impact on activities of daily living and work disability. The need for external home help and disability are increased for those patients with DUs.

Hand vascular involvement assessed by magnetic resonance angiography in systemic sclerosis.

OBJECTIVE: Impairment of the microcirculation is a cardinal feature of systemic sclerosis (SSc). Magnetic resonance angiography (MRA) has improved the assessment of vascular lesions of the hand. The aim of this study was to evaluate vascular abnormalities in the hands of patients with SSc, using MRA. METHODS: Thirty-eight patients with SSc were compared with 7 healthy subjects and 7 patients with rheumatoid arthritis. Among patients with SSc, the mean +/- SD age was 52 +/- 14 years, the mean +/- SD Health Assessment Questionnaire (HAQ) score was 0.9 +/- 0.8, and the mean +/- SD systolic pulmonary artery pressure (PAP) was 32.2 +/- 8.4 mm Hg. Ten patients had a history of digital ulcers. The MRA protocol consisted of 4 successive acquisitions, each lasting 52 seconds, of 3-dimensional coronal cross-sectional images after gadolinium injection. The primary criteria were distality and quality of arterial opacification, avascular areas, and venous return. RESULTS: Thirty-five of the patients with SSc (92%) had at least 1 true digital artery that did not reach the first phalanx at the initial arterial analysis, and 23 patients (61%) had > or =4 damaged arteries. Twenty-eight patients (74%) had thin arteries, and 20 patients (53%) had >1 avascular area. Nearly all patients (35 of 38 [92%]) had abnormal venous return, and a lack of visible venous return was observed in 16 patients (42%). Results for all of the control subjects were considered normal. Digital ulcers were more frequently observed in patients with SSc who had > or =4 damaged proper digital arteries compared with other patients (P = 0.003), the HAQ score was associated with thin-caliber arteries (P = 0.04), and systolic PAP was associated with tissue enhancement secondary to ischemia (P = 0.04). CONCLUSION: These results show the substantial vascular involvement in SSc. Lesions were diffuse and involved both arterial and venous vessels of small caliber as well as the microcirculation.

Meralgia paresthetica: clinical and electrophysiological diagnosis in 120 cases.

We report the results of clinical and electrophysiological examinations in 131 cases of meralgia paresthetica (MP) among 120 unselected patients, 69 men and 51 women, aged 15-81 years. All patients experienced permanent or intermittent pain, and all but one had permanent sensory impairment of the thigh. The lateral aspect of the thigh was solely involved in 88 cases and the anterior aspect was also or exclusively involved in 32 cases. The right thigh was involved 62 times and the left 58 times. Symptom duration varied from 2 weeks to 20 years. The initial diagnosis was meralgia paresthetica in 47 cases (39%), root disease in 35 cases, and osteoarthritis in 6 cases; no diagnosis was proposed in the 32 remaining cases. Two cases had undergone previous spine surgery for disk herniation, with no benefit. A precise cause could explain the lateral femoral cutaneous nerve (LFCN) lesion in 46 cases, the other 74 cases being considered idiopathic (25% of patients were obese). Only one case required surgery to relieve symptoms. LFCN conduction was studied orthodromically, distally from the anterior superior iliac spine. The side-to-side amplitude ratio (ssRatio) was greater than 2.3 in 118 of 120 patients (98.3%) and was a better index to confirm a lesion of the LFCN than SNAP amplitude, which was abnormal (less than 3 microV) in 88 cases (73.3%). Only two of the 11 bilateral cases had an ssRatio lower than 2.3 (they were both 2.0). An ssRatio of 2.3 or more and a SNAP amplitude lower than 3 microV provided a specificity of 98.75% or more. The mean axonal loss was 88%. These clinical and electrophysiological data highlight the central role the neurophysiologist should play in diagnosing MP by means of an LFCN conduction study.