Assessment of disease severity and prognosis Medsger, Jr, T A; Bombardieri, S; Czirjak, L ...
Clinical and experimental rheumatology,
2003, Volume:
21, Issue:
3 Suppl 29
Journal Article
Peer reviewed
The Subcommittee members initially agreed on the concepts of disease activity, damage and severity, defining severity as the total effect of disease on organ function. It was decided to start with ...the assessment of severity using the Medsger's severity scale. A revised version of this scale was constructed. The rationale for the exclusion of other variables was provided.
...because both KTSS and mRTSS are based on a three point scale, we assigned a score to each of the 17 areas specified by the modified Rodnan system; this score was drawn from the data gathered for ...the evaluation of the KTSS. ...we ruled out the values relating to the neck, back, and buttocks, and for the fingers, where the Kahaleh system requires two scores-that is, distal and proximal), we used the higher score.
The minor salivary gland biopsy (MSGB) is widely considered an important component of the diagnostic algorithm of primary Sjögren's syndrome (pSS) and is mentioned in all the classification criteria ...sets for the disease. The aim of this study, coordinated by the Italian Society of Rheumatology, was to verify the inter-observer agreement on the evaluation of MSGB among different experienced Italian rheumatologic centres, in order to better standardise the diagnostic methodology.
Seven centres participated in the study, providing a total of 50 MSGB samples. Each center blindly classified all the samples according to the Chisholm and Mason (CM) grading. The results were collected and analysed.
The inter-observer agreement was satisfactory when the samples were stratified as consistent and non-consistent with the final diagnosis of pSS (median κ =0.75; mean κ =0.70). Nonetheless, significant discrepancies in the histopathologic evaluation of MSGB emerged when the agreement was assessed on the single scores. Considering the modal CM grading for each sample as the correct grading, upon re-examination, a potential bias in the final clinical diagnosis was detected in 7 out of 50 samples.
This study has shown significant discrepancies in the evaluation of MSGB among different rheumatologic centres in the same country. Greater standardisation of the procedure is clearly necessary, both to improve the diagnostic performance and scientific communication.
Background:
The most important side effect of radioiodine (
131
I) therapy is sialoadenitis and xerostomy.
Aim:
To evaluate by ultrasound (US) parotid and submandibular glands after
131
I therapy for ...differentiated thyroid cancer (DTC).
Patients:
Seventy-six subjects thyroidectomized for DTC submitted to salivary glands US examination. Forty-three of them had been previously treated with
131
I: 22 with 1.11 GBq (30 mCi) for remnant ablation, and 21 with higher doses up to 44.4 GBq (1200 mCi) for metastases. Thirty-three subjects studied before
131
I therapy served as controls. Parotid and submandibular volume, homogeneity, and echogenicity were determined.
131
I-treated patients filled a questionnaire about sialoadenitis symptoms.
Results:
Parotid gland volume was significantly higher in treated patients (28.3±16.2 ml) than in untreated patients (20.7±10.4 ml, p=0.0154) and related to the time from last
131
I therapy. Three had parotid volume <1.5 ml and complained severe xerostomy. Submandibular gland volume was similar in treated (11.2±7.6 ml) and untreated patients (8.6±4.2 ml, p=0.0602). Homogeneity and echogenicity were similar in treated and untreated patients. Sialoadenitis symptoms were reported in 26% and were related to the
131
I cumulative dose. Symptoms were not related to gland volume. Hypoechogenicity and inhomogeneity of the parotids were more frequent in patients with salivary stickiness.
Conclusion:
Parotid, but not submandibular, volume is increased after
131
I treatment depending on the received activity and the time from irradiation but not on sialoadenitis symptoms. Xerostomy is associated to gland atrophy at US.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Objective: To further assess the construct validity of the three European Scleroderma Study Group (EScSG) preliminary activity indices for systemic sclerosis (SSc): for SSc as a whole, for diffuse ...SSc (dcSSc), and for limited SSc (lcSSc). Methods: 30/290 SSc clinical charts collected for the EScSG study used to develop activity criteria for SSc were selected and sent to four clinical experts in SSc. The experts ranked the charts from 1 to 30 (1=lowest activity, 30=highest activity). The relationships among the ranks given by each investigator and each of the three scores, and between any two of the ranks were investigated. Results: A consistently significant correlation (rs=0.530–0.712) was found between the ranks given by each of the four investigators and the index for the entire patient group. A similar level of agreement was detected between each couple of the four experts (rs=0.428–0.720). Moreover, the ranks given in patients with an index >3 were significantly higher than those given for patients with an index ⩽3. This cut off point had previously been shown to best discriminate patients with active disease. Conclusions: Of the originally developed activity indexes, the whole series index has been externally validated. The index comprises the first preliminary, but necessary, groundwork to improve the concept of disease activity in SSc, which is still ill defined. It can be used as a preliminary activity index in clinical investigational studies.
Extracorporeal photopheresis (ECP) has been considered an efficient dendritic cell (DC) therapy, used for treating both T cell malignancy, as well as T cell-mediated diseases. During the ECP ...procedure leucocytes are exposed to photoactivable agent 8-methoxypsolaren (8-MOP) and ultraviolet (UV) A radiation (PUVA) prior to reinfusion. Despite its clinical efficacy the mechanism of action remains elusive. As it has been reported that ECP might promote the differentiation of monocytes into immature DCs, we investigated the effects of UVA light (2 J/cm²) and 8-MOP (100 ng/ml) on in vitro monocyte-to-DC differentiation from normal donors. DCs were generated from human purified CD14⁺ cells. Because monocytes are killed by PUVA and taking into account that only 5-10% of circulating mononuclear cells are exposed to PUVA during the ECP procedure, we developed an assay in which 10% of PUVA-treated monocytes were co-cultured with untreated monocytes. We first demonstrate that the presence of 10% apoptotic cells and monocyte activation were not enough to induce monocyte differentiation into DCs. Adding cytokines to our culture system, we obtained immature DCs characterized by significantly higher phagocytic activity and human leucocyte antigen D-related (HLA-DR) expression. These DCs preserved the capacity to be activated by lipopolysaccharide, but showed a reduced capacity to induce allogeneic T cell proliferation when first co-cultured with 10% of PUVA-treated cells. Our experimental design provides a novel insight into the real action of 8-MOP and UVA light on dendritic cell biology, suggesting an additional mechanism by which 8-MOP and UVA light exposure may influence immune responses.
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BFBNIB, DOBA, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, SIK, UILJ, UKNU, UL, UM, UPUK
OBJECTIVE To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. METHODS Investigators from 19 European centres completed a standardised ...clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0–10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity (“inactive to moderately active” or “active to very active” disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. RESULTS A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including Δ-factors—that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) Δ-cardiopulmonary (4.0), Δ-skin (3.0), Δ-vascular (2.0), and Δ-articular/muscular (1.0) for patients with dSSc; (b) Δ-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), Δ-cardiopulmonary (1.5), Δ-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) Δ-cardiopulmonary (2.0), Δ-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5), Δ-vascular (0.5), arthritis (0.5), Tlco <80% (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for “active to very active” patients. CONCLUSIONS Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.
Treatment with 131I for differentiated thyroid cancer
may give a follicle-damaging radiation dose to the ovaries. This damage
to the ovarian function could shorten the fertile life span and advance
...the natural menopause. To address this issue, we studied
retrospectively the menopausal age of 130 women treated with
131I for differentiated thyroid cancer in our institution
from 1974–1993. The menopausal age of women treated with
131I for differentiated thyroid cancer after
total thyroidectomy and subjected to suppressive
l-T4 therapy was compared with the
menopausal age of a control group including 127 goitrous women who were
treated with suppressive l-T4 for a
comparable period of time. The cumulative therapeutic
131I dose to cancer patients ranged from
1,110–40,700 MBq (mean ± sd, 5,308 ± 5,483
MBq; median, 3700 MBq). All patients chosen for the study were younger
than 45 yr when first treated (i.e. first administration of
131I and l-T4 for
cancer patients, and institution of l-T4therapy for goitrous patients), and older than 45 yr at the end of the
study period. The menopausal status of both groups was assessed from
the clinical records and compared using Kaplan-Meier survival analysis.
The menopausal age of cancer women treated with 131I
and suppressive l-T4 therapy was less
than that of goitrous patients treated with suppressive
l-T4 therapy (P <
0.001). We could not detect any relationship between menopausal age and
the age at the first or last 131I dose or to the
cumulative 131I dose received. These data indicate
that 131I treatment is probably associated with
an earlier ovarian failure in thyroid cancer patients. Conceivably, the
ovarian irradiation by 131I might contribute to the
process of the follicular atresia, thus inducing earlier menopause.
Objectives: Mixed cryoglobulinaemia (MC) is a chronic small-vessel vasculitis. Shortly after the discovery of hepatitis C virus (HCV) in 1989, an association between HCV infection and MC was being ...increasingly reported, suggesting the potential pathogenetic implication of HCV in most of the cases that had been previously diagnosed as essential MC. A number of studies have pointed out prognostic factors linked to mortality in this disorder. None of them, however, have clarified the impact of HCV discovery on the natural history of the disease. The aim of the present study was to evaluate mortality in MC after the discovery of HCV infection.
Methods: We retrospectively collected clinical and serological data in 70 unselected HCV-positive patients being followed up at our unit from 1990. Clinical and prognostic factors linked to poor outcome were evaluated.
Results: Chronic hepatitis, renal involvement, and intestinal vasculitis were the most frequent causes of death.
Conclusion: Compared to other series, the outcome in our MC seemed to be better. Factors linked to a poor outcome were renal involvement, widespread vasculitis, male sex, and cryocrit.
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DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Aim: To determine whether the European Consensus Lupus Activity Measurement Index (ECLAM) can be used to evaluate disease activity in patients retrospectively from the data provided in their clinical ...charts.
Methods: The ECLAM score was calculated twice in a series of 64 consecutive SLE patients: first for each patient during the course of a standard clinical evaluation (direct-ECLAM), and then one to two weeks later solely on the basis of the data provided in the patient's clinical chart (chart-ECLAM). The scorings for each patient were performed by two different assessors.
Results: The direct-ECLAM and chart-ECLAM scores were highly correlated (Spearman's rank correlation coefficient = 0.86). The regression line was not significantly different from the identity line (t-test). The Pearson's coefficient was 0.88. The interobserver variability of the chart-ECLAM showed a low inter-rater variability.
Conclusion: ECLAM could represent a valid and reliable instrument for the retrospective analysis of disease activity in SLE patients.
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DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
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