Objective
To assess patient priorities concerning disability in systemic sclerosis (SSc).
Methods
A total of 150 SSc patients (22 men) fulfilling the American College of Rheumatology and/or LeRoy and ...Medsger criteria for SSc were evaluated by the McMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR), Karnofsky performance status (KPS), Cochin Hand Function Scale, Health Assessment Questionnaire (HAQ), Hospital Anxiety and Depression Scale, Mouth Handicap in SSc (MHISS) scale, and global perception regarding their health status. Correlations between scores were analyzed using Spearman's coefficient. Logistic regression analysis was used to determine factors associated with patients' global perception of their health.
Results
Of the patients investigated, 81 (54%) had limited cutaneous SSc, 65 (43.3%) diffuse SSc, and 4 (2.7%) limited SSc. The 3 disability domains most often cited were walking (82 patients 54.6%), housekeeping (67 patients 44.6%), and sport activities (59 patients 39.3%). The MACTAR score correlated moderately with KPS (r = 0.58) but only weakly with the HAQ score (r = 0.38). In multivariate analysis, 2 factors were associated with patients' negative global perception of their health status: KPS (odds ratio OR 1.07, 95% confidence interval 95% CI 1.00–1.15) and MHISS score (OR 0.93, 95% CI 0.88–0.99).
Conclusion
For assessing SSc patient priorities concerning disability, the MACTAR has acceptable construct validity. Its weak correlation with the HAQ suggests that it adds useful information on disability.
Background: Pulmonary arterial hypertension (PAH) is a frequent cause of morbidity and mortality in patients with systemic sclerosis
(SSc). PAH is generally considered to be a late complication of ...limited cutaneous SSc. This study identified and investigated
a subset of SSc patients with early-onset PAH.
Methods: Clinical and hemodynamic data at the time of diagnosis were collected retrospectively for 78 consecutive patients with PAH
associated with SSc. PAH diagnosed within 5 years of the first non-Raynaud phenomenon symptom of SSc was considered to be
an early-onset complication. PAH diagnosed > 5 years following SSc diagnosis was considered to be a late complication.
Results: PAH occurred a mean (± SD) duration of 6.3 ± 6.6 years after the diagnosis of SSc (median delay, 4.0 years; 95% CI, 2.88
to 6.0 years). Early-onset PAH was diagnosed in 43 patients (55.1%), and late-onset PAH was diagnosed in 35 patients (44.9%).
Patients with early-onset PAH were older at SSc diagnosis than patients with late-onset PAH (mean age, 58.0 ± 12.5 vs 46.6
± 12.9 years, respectively; p = 0.0002). No differences in age at the time of PAH diagnosis, or in SSc subtype (limited vs
diffuse; anticentromere vs anti-Scl70 antibodies), were observed between onset subgroups. At diagnosis, early-onset PAH was
more severe than late-onset PAH, with a lower cardiac index (2.4 ± 0.6 vs 2.8 ± 0.6 L/min/m 2 , respectively; p = 0.005) and greater total pulmonary resistance (1,708 ± 777 vs 1,341 ± 530 dyne · s · cm â5 /m 2 , respectively; p = 0.02). Mortality at 3 and 5 years was comparable between subgroups.
Conclusions: In contrast to the expected scenario, early-onset PAH occurred in approximately half of SSc patients. Early-onset PAH was
as frequent among patients with diffuse SSc as those with limited SSc. Annual screening for PAH should be implemented immediately
after SSc diagnosis for all patients.
To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE).
We queried the European League ...Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes.
Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9-82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1-0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2-21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1-113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01).
GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.
Background Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or ...scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD. Methods Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure mPAP ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg). Results As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (D lco) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio HR, 2.44; P = .04) and lower D lco (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group. Conclusions Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower D lco and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.
Until recently, systemic sclerosis (SSc) was thought to spare the central nervous system (CNS). Neurological symptoms secondary to CNS involvement are very rare in SSc patients. Conversely, the ...prevalence of depression in SSc patients ranges from 17 to 65% and is much higher than that observed in the general population. Cognitive impairment has been reported in SSc patients, but these findings require confirmation in further studies. Brain calcifications and hyperintense white matter signals (leukoaraiosis) have been documented in SSc patients at a higher incidence than in control populations. Severe leukoaraiosis lesions seem to be associated with severe vascular manifestations in SSc. If morphological CNS abnormalities could be linked to neuropsychiatric manifestations, it would be possible to identify neuropsychiatric scleroderma as we can now identify neuropsychiatric systemic lupus erythematosus.
Les patients atteints de vascularite systémique associée aux ANCA (VSAA) souffrent généralement d’arthralgies et, parfois, de polyarthrite durant les poussées. Bien que le facteur rhumatoïde (FR) ...puisse être détecté chez 37 à 50 % des patients VSAA, les anticorps antipeptides cycliques citrullinés (anti-CCP) sont rares. Nous décrivons dans cet article les caractéristiques cliniques de cinq patients atteints de vascularite ANCA-positives qui présentaient anticorps anti-CCP persistants et/ou à taux élevés et d’arthralgies récidivantes non destructrices et de polysynovites, indépendamment de l’évolution de la vascularite et en l’absence d’évidence de PR. Chez deux d’entre eux, cependant on pensait initialement à une PR plutôt qu’à une polyangéite microscopique (PAM). Au moment du diagnostic de la VSAA, tous présentaient une atteinte rénale et trois d’entre eux, des hémorragies alvéolaires. Avec un suivi médian de 30 mois, un patient a eu des poussées de vascularite, précédées d’une polysynovite, et les autres ont eu des arthralgies récidivantes et une polysynovite, alors que leur vascularite demeurait en rémission. Aucun de ces patients ne présentait d’arthrite destructrice à la radiographie. Nous discutons ici la difficulté du diagnostic chez ces patients avec ANCA et anti-CCP et présentant des manifestations articulaires prédominantes. Les patients VSAA avec des anticorps anti-CCP peuvent présenter des polysynovites et des polyarthrites non destructrices avant les poussées de vascularite, mais également indépendamment de l’évolution de la vascularite, ce qui est moins commun au cours des VSAA. Ces patients constituent un petit sous-groupe au sein des VSAA.
Systemic Sclerosis-Associated Myopathy RANQUE, BRIGITTE; AUTHIER, FRANÇOIS-JÉRÔME; BEREZNE, ALICE ...
Annals of the New York Academy of Sciences,
June 2007, Volume:
1108, Issue:
1
Journal Article
Peer reviewed
: Skeletal muscle involvement is a common feature in systemic sclerosis (SSc) because muscle weakness is found in up to 90% SSc patients when systematically assessed. Muscle clinical, biological, ...and electromyographic features are similar to those of polymyositis or dermatomyositis, except for a higher proportion of mild symptoms. SSc‐associated myopathy is more prevalent in diffuse SSc and is also associated with cardiomyopathy. The pathophysiological process leading to SSc‐associated myopathy is likely to be complex, given the heterogeneity of pathological muscle findings, including stigma of microangiopathy, and also inflammatory infiltrate in about half of the cases and interstitial fibrosis. Conflicting results have been reported regarding the correlation between clinicobiological presentation and pathological muscle features, nevertheless there is a general agreement that histologically proven inflammatory myopathies usually regress under high‐dose corticosteroid therapy, or even low dose in case of positive anti‐PM/Scl antibody. In contrast, noninflammatory myopathies often result in milder clinical expression but do not respond to immunosuppressive treatment.
Published studies on birdshot chorioretinopathy (BCR) did not provide definitive information on possibly associated extraocular manifestations.
Single-center cross-sectional analysis of extraocular ...manifestations in a cohort of patients with BCR.
Since 2002, 118 patients (45 men, 73 women) were enrolled. Their mean age was 51.5 years at diagnosis. The most common features of their medical histories were: hypertension (32 patients), drug allergy (19), sinusitis (17), thyroid disease (12), otitis media (11), asthma (11); diabetes (10); cancer (8); psoriasis (5); monoclonal gammopathies (3). At the time of disease onset, arthralgias were noted in 23, ENT manifestations in 26, Raynaud's phenomenon in 6, headaches in 10, psoriasis in 3 others. Between diagnosis and cross-sectional evaluation visits, only the frequency of hypertension has increased significantly (11 additional patients).
No predominant extraocular manifestation of BCR was identified in our patients. Their ongoing follow-up may yet discern whether BCR is definitively eye-restricted.
La choriorétinopathie de
birdshot est une uvéite postérieure chronique associée à l’allèle A29 du CMH de classe I et fréquemment à une vascularite rétinienne angiographique, suggérant qu’il puisse s’agir d’une maladie auto-immune. Les séries déjà publiées, toutes rétrospectives, comportent peu de patients et n’ont pas permis d’étudier précisément la fréquence de toutes les éventuelles manifestations extra-oculaires.
Enquête clinique transversale d’une cohorte monocentrique de patients atteints de choriorétinopathie de
birdshot. Les patients ont été vus et interrogés de manière systématique par des internistes. Les données ont été recueillies à l’aide d’un formulaire standardisé, déjà utilisé pour plusieurs études dans les vascularites systémiques.
Depuis 2002, 118 patients ont été inclus, âgés de 51,5
±
16 ans au diagnostic. Parmi leurs principaux antécédents, on notait une HTA (32 patients), des allergies médicamenteuses (
n
=
19), des sinusites (
n
=
17), des pathologies thyroïdiennes (
n
=
12), des otites moyennes (
n
=
11), un asthme (
n
=
11), un diabète (
n
=
10), un cancer (8), un psoriasis (5) et/ou une gammapathie monoclonale (3). Au début de la maladie, 23 patients avaient des arthralgies, 26 des manifestations ORL, notamment une baisse d’acuité auditive (
n
=
8) et 6 un syndrome de Raynaud. Entre le diagnostic et la visite d’évaluation, 11 patients supplémentaires avaient une HTA, en majorité liée à la prise de ciclosporine.
La fréquence des manifestations extra-oculaires au cours de la choriorétinopathie de
birdshot ne paraît pas plus importante que dans la population générale. Le suivi de cette cohorte permettra de confirmer s’il s’agit bien d’une pathologie ophtalmologique limitée.
To assess the value of major histocompatibility complex (MHC) class II antigen (HLA-DR) expression to distinguish anti-synthetase myopathy (ASM) from dermatomyositis (DM).
Muscle biopsies from ...patients with ASM (n = 33), DM without anti-synthetase antibodies (ASAb) (n = 17), and normal muscle biopsy (n = 10) were first reviewed. ASAb included anti-Jo1 (26/33), anti-PL12 (4/33), anti-PL7 (2/33), and anti-EJ (1/33). Immunohistochemistry was performed for MHC-I/HLA-ABC, MHC-II/HLA-DR, membrane attack complex (C5b-9), neural cell adhesion molecule (NCAM)/CD56 expression, and inflammatory cell subsets. Twenty-four ASM and 12 DM patients from another center were added for HLA-DR evaluation.
Ubiquitous myofiber HLA-ABC expression was equally observed in ASM and DM (93.9% vs 100%, NS). In contrast, myofiber HLA-DR expression was found in 27/33 (81.8%) ASM (anti-Jo1: 23/26, 88.5%; others: 5/7, 71.4%) vs 4/17 (23.5%) DM patients (p < 0.001). HLA-DR was perifascicular in ASM, a pattern not observed in DM. In addition, C5b-9 deposition was observed on sarcolemma of non-necrotic perifascicular fibers in ASM, while, in DM, C5b-9was mainly detected in endomysial capillaries. CD8 cells were more abundant in ASM than in DM (p < 0.05), and electively located in perimysium or in perifascular endomysium. HLA-DR expression correlated positively with the CD8+ cells infiltrates. Strictly similar observations were made in the confirmatory study.
ASM is characterized by strong myofiber MHC-II/HLA-DR expression with a unique perifascicular pattern, not described so far. HLA-DR detection must be included for routine myopathological diagnosis of inflammatory/dysimmune myopathies. HLA-DR expression in ASM may indicate a specific immune mechanism, possibly involving IFNγ.
Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking.
We conducted a multicenter, ...retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner.
CM were more frequent in EGPA (53.0%) and MPA (51.9%) than in GPA (36.7%). Lesions more frequently associated with GPA were oral ulcers (4.6% vs. 2.5% in EGPA and 0.3% in MPA), while pyoderma gangrenosum and palpebral xanthoma were specific to GPA. Lesions associated with MPA were segmentary edema (19.5% vs. 12.7% in EGPA and 4.3% in GPA) and livedo (12.4% vs. 0.5% and 2.6%, respectively), whereas those associated with EGPA were urticarial lesions (11.5% vs. 1.9% in GPA and 3.5% in MPA) and nodules (12,2% vs. 8.9% in GPA and 4.7% in MPA). In GPA, CM patients had more frequent vasculitis than granulomatous phenotype, and poorer relapse-free and overall survival. Pathological analysis showed vasculitis and/or granulomatous infiltrates in 87.5% of GPA, in 61.1% of EGPA and in all MPA. Vasculitis was more frequently observed in purpura and nodules, while granulomas were differently located and organized within vessels or interstitium according to the type of lesions.
Each AAV seemed to be associated with a peculiar pattern of cutaneous lesions. CM are associated with poorer prognosis in GPA. Clinical-pathological correlations showed no specific feature of each AAV, whereas granulomatous infiltrates differ according to the type of lesions.
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