ABSTRACT Background A great challenge in clinical practice is the differential diagnosis between ... more ABSTRACT Background A great challenge in clinical practice is the differential diagnosis between rheumatic inflammatory diseases, such as Adult Onset Still’s disease (AOSD), and sepsis. Indeed, these conditions share several clinical and laboratory findings. Recent studies have investigated the cytokine profile in patients with AOSD and sepsis detecting elevated serum levels of different cytokines, including interleukin (IL)-18. This cytokine plays a pivotal role in AOSD, by driving the inflammatory process. Objectives To investigate IL-18 serum levels in AOSD and to assess whether IL-18 could be used as a biomarker to discriminate between patients with AOSD and sepsis. Methods We measured IL-18 serum levels by a commercial ELISA kit (Immuno Pharmacology Research, Italy), in a cohort of patients with AOSD (diagnosed according to Yamaguchy criteria) and in patients sepsis (diagnosed according to American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference). In AOSD, disease activity was evaluated with Rau’s criteria, and patients were defined “active” if they scored ≥4. Area under the receiver operating characteristic curve (ROC-AUC) analysis was used to evaluate the diagnostic utility of the IL-18. Results Thirty patients with AOSD (F 18, M 12, mean age 39.6 years, range 18-69) and 7 patients with sepsis (F 4 M 3, mean age 35.1 years, range 30-55) were enrolled. Mean IL-18 serum level in AOSD patients [1298.7 pg/ml (range 0–6015)] was significantly higher from mean IL-18 serum level in sepsis [113.5 pg/ml (range 52–328), P=0.008]. ROC-AUC analysis of the serum concentration of IL-18 indicated that it was significantly diagnostic of AOSD, especially when active. The ROC-AUC analysis for the serum level of the IL-18 between patients with AOSD and sepsis was 0.712. At a cutoff point of 179 pg/ml, corresponding to the greatest sum of specificity and sensitivity, the specificity was 69.7% and the sensitivity was 87.5% for detection of AOSD (likelihood 2.89). When disease activity was scored according to Rau’s criteria, 21/30 (70%) patients were identified as active. These patients showed mean IL-18 serum levels (1793 pg/ml, range 0-6015) significantly higher than patient with “non active” disease (145.5 pg/ml, range 0-685, P=0.0039), as well as higher than sepsis (P=0.007). The ROC-AUC analysis for the serum level of the IL-18 between active patients with AOSD and sepsis was 0.845. At a cutoff point of 223 pg/ml, the specificity was 87.5% and the sensitivity was 80.9% for detection of AOSD (likelihood 6.48). Conclusions A significant difference in IL-18 serum levels between patients with AOSD and sepsis was found, especially in those patients with active disease. This evidence may confirm the potential utility of IL-18 in the differential diagnosis between AOSD and sepsis. Disclosure of Interest None Declared
ABSTRACT Background Although muscle pain is relatively frequent in primary Sjogren Syndrome (pSS)... more ABSTRACT Background Although muscle pain is relatively frequent in primary Sjogren Syndrome (pSS), a frank myositis is rare and reported only in 2.5-11% of patients. The most frequent symptoms are muscular weakness and myalgias and diagnosis is based on clinical, laboratory and histologic evaluation. Objectives To describe the prevalence and course of myositis in a multicenter cohort of patients with pSS. Methods Clinical and laboratory data from patients with a diagnosis of pSS were retrospectively collected. The clinical charts of patients with symptoms and/or signs of inflammatory myositis were carefully reviewed. Results One thousand one hundred seventy patients with pSS (52M, 1118 F, mean age 57.3 years; range 17–89 years) were considered. Mean age at diagnosis was 51±14 years and the mean follow up from diagnosis was 5.6 years (range 0–42 years). Ten female patients (0.85%) presented invalidating muscular weakness in the upper and lower limbs and/or severe myalgias. In 8/10 patients an increase of Creatine Phosphokinase (CK) serum level was present. A possible iatrogenic effect was excluded; in one case myositis started under statin therapy but didn’t disappear after drug discontinuation. In the 10 patients, mean age at pSS diagnosis was 47.4 years (range 14–68), while mean age of myositis diagnosis was 50.6 years (range 23–69). In six out of ten, muscular and sicca symptoms had occurred together at onset. Diagnosis of myositis was supported by clinical evidences and by the presence of increased CK serum levels. In 7/10 patients muscle biopsy was performed. In all biopsies signs of inflammation were present. In 1/7 patients (14.2%) the biopsy was typical of an inclusion body myositis, while in the remaining cases histological aspects of Dermatomyositis and Polymyositis (PM) were detected. Eight out of ten patients presented at least three criteria for inflammatory myopathy (according to Bohan and Peter criteria, 1975) suggesting a possible diagnosis of a true overlap syndrome. In one of these patients serum anti Jo-1 and anti RNP antibodies were detected. Six patients (60%) were successfully treated with DMARDs, while four (40%) did not respond. Thus, two were treated with IvIg, one showing complete remission of myositis, and the remaining two non-responder patients were treated with Rituximab, one showing complete remission. Conclusions Our retrospective study shows a lower prevalence of myositis in pSS than previously reported. Muscle involvement can occasionally represent the first symptom at disease onset. An overlap syndrome with inflammatory myopathies, above all PM, should be considered. Different therapeutic approaches can be used: DMARDs are effective in the majority of the patients, while IvIg and Rituximab may represent an available alternative therapeutic options in non-responder patients. Disclosure of Interest None Declared
Clinical and experimental rheumatology, Jan 19, 2015
In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequ... more In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was per...
To assess the quality of sexual life of women with primary Sjögren syndrome (pSS) and to identify... more To assess the quality of sexual life of women with primary Sjögren syndrome (pSS) and to identify its correlations with disease activity and damage, quality of life, and mood disorders. The quality of sexual life of 24 women with pSS was assessed with the Female Sexual Function Index (FSFI). Twenty-four healthy women, matched by age and hormonal status, were enrolled as controls. Mood disorders and quality of life were investigated using the Hospital Anxiety and Depression Scale (HADS) and the Medical Outcomes Study Short Form-36. Patients underwent a gynecological visit with vaginal pH measurement, cervicovaginal swabs, and Pap smears. Disease activity and damage were assessed by the European League Against Rheumatism Sjögren syndrome disease activity and damage indexes. Patients with pSS showed a pathological mean FSFI score (19.1 ± 7.33) significantly different from controls (p = 0.004), both in menstruating women (p = 0.006) and in menopausal women (p = 0.03). Major differences between the 2 groups were detected in dyspareunia (p < 0.005), lubrication (p = 0.006), desire (p = 0.004), and arousal (p = 0.018). The FSFI score was inversely correlated with age (p = 0.008) and anxiety HADS (p = 0.031). No early anatomical changes, swabs, and Pap smear alterations were revealed in patients with pSS; however, vaginal pH was higher than normal in premenopausal patients (6.0 ± 0.77). Both premenopausal and postmenopausal women with pSS have a worse sexual quality of life. We reported a greater prevalence of dyspareunia that is statistically significant when compared with controls. The FSFI could be a useful tool to assess this topic, but has been neglected in the care of patients with pSS heretofore.
Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life... more Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Chronic widespread musculoskeletal pain and fatigue are the clinical hallmarks of fibromyalgia (FM), a clinical entity which can be associated to connective tissue disease. The aim of the present study was to assess the prevalence of FM syndrome, fatigue and widespread pain in SLE and pSS patients and to evaluate the contribution of inflammatory disease and FM on those constitutional symptoms. Fifty SLE and 50 pSS patients were enrolled in the study. Patients rated fatigue, pain, and disease activity using a 100-mm visual analogue scale and completed the Health Assessment Questionnaire and the Fibromyalgia Impact Questionnaire. Zung depression and anxiety scales were used to quantify mood disorders. Tender points were evaluated using an algometer. Disease activity score as evaluated for each SL...
To determine the clinical and laboratory differences between cryoglobulinaemic and hypergammaglob... more To determine the clinical and laboratory differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome (pSS), in a large Italian multicentre cohort. Patients were selected according to the following criteria: fulfilling the American-European classification criteria for pSS, serum cryoglobulin and gammaglobulin levels evaluated, and lack of hepatitis C virus (HCV) infection. Multinomial analyses were performed by distinguishing three groups of pSS: (i) purpura associated with cryoglobulinaemic vasculitis (CV), (ii) purpura associated with hypergammaglobulinaemic vasculitis (HGV), and (iii) pSS patients without purpura (pSS controls). Patients with purpura but without cryoglobulins or hypergammaglobulinaemia were excluded. A total of 652 patients were enrolled in this study. Group 1/CV comprised 23/652 patients (3.53%), group 2/HGV 40/652 patients (6.13%), and group 3/pSS controls 589/652 (90.34%). The three groups were found to be signifi...
In primary Sjögren&am... more In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested…
To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS). An o... more To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS). An obstetric history of 36 women with established diagnosis of pSS at pregnancy was obtained from a multicenter cohort of 1075 patients. In a subgroup case-control analysis, 12 deliveries in patients with pSS were compared with 96 control deliveries. Thirty-six women (31 with anti-SSA/Ro and/or anti-SSB/La antibodies) with an established diagnosis of pSS had 45 pregnancies with the delivery of 40 newborns. Two miscarriages, 2 fetal deaths, and 1 induced abortion were recorded. Mean age at the first pregnancy was 33.9 years; mean number of pregnancies was 1.25; 18/40 (45%) cesarean births were delivered; mean pregnancy length was 38.5 weeks (range 32-43), with 6 preterm deliveries. The mean Apgar score at 5 min was 8.9, mean birthweight was 2920 g (range 826-4060 g). Congenital heart block (CHB) occurred in 2/40 (5%) newborns. The reported rate of breastfeeding for at least 1 month was 60.5%. In 4/40 pregnancies (10%) a flare of disease activity was observed within a year from delivery. In the case-control subgroup analysis, 12 deliveries were compared with 96 controls and no significant differences were found. Patients with pSS can have successful pregnancies, which might be followed by a mild relapse. CHB was the only cause of death for offspring of mothers with pSS.
The differential diagnosis between rheumatic diseases and infectious conditions is a great challe... more The differential diagnosis between rheumatic diseases and infectious conditions is a great challenge in clinical practice. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory syndrome that shares several clinical and laboratory variables with sepsis. Interleukin (IL)-18 is overexpressed in AOSD, suggesting a possible role as a disease biomarker. The aim of our study was to detect IL-18 serum levels in a cohort of patients with AOSD and sepsis and to address its possible role as a biomarker for differential diagnosis. A group of unselected patients with AOSD diagnosed according to the Yamaguchi criteria and consecutive patients with sepsis diagnosed according to the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria were enrolled. The clinical and laboratory data were collected. In the AOSD group, disease activity was assessed by Pouchot's and Rau's criteria. IL-18 serum levels were detected by ELISA. Thirty-nine patients with AOSD and 18 patients with sepsis were enrolled. Two out of 18 patients with sepsis (11.1%) also fulfilled the Yamaguchi criteria. A significant difference was found in IL-18 serum levels between patients with active and inactive disease (p < 0.001), and it positively correlated with disease activity (p = 0.0003), ferritin serum level (p = 0.016), and erythrocyte sedimentation rate (p = 0.041). IL-18 was significantly increased in patients with AOSD when compared with sepsis (p = 0.014). For a cutoff of 148.9 pg/ml, this test had a specificity of 78.3% and a sensitivity of 88.6%. We have demonstrated that IL-18 can be a biomarker for differential diagnosis between AOSD and sepsis.
ABSTRACT Background A great challenge in clinical practice is the differential diagnosis between ... more ABSTRACT Background A great challenge in clinical practice is the differential diagnosis between rheumatic inflammatory diseases, such as Adult Onset Still’s disease (AOSD), and sepsis. Indeed, these conditions share several clinical and laboratory findings. Recent studies have investigated the cytokine profile in patients with AOSD and sepsis detecting elevated serum levels of different cytokines, including interleukin (IL)-18. This cytokine plays a pivotal role in AOSD, by driving the inflammatory process. Objectives To investigate IL-18 serum levels in AOSD and to assess whether IL-18 could be used as a biomarker to discriminate between patients with AOSD and sepsis. Methods We measured IL-18 serum levels by a commercial ELISA kit (Immuno Pharmacology Research, Italy), in a cohort of patients with AOSD (diagnosed according to Yamaguchy criteria) and in patients sepsis (diagnosed according to American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference). In AOSD, disease activity was evaluated with Rau’s criteria, and patients were defined “active” if they scored ≥4. Area under the receiver operating characteristic curve (ROC-AUC) analysis was used to evaluate the diagnostic utility of the IL-18. Results Thirty patients with AOSD (F 18, M 12, mean age 39.6 years, range 18-69) and 7 patients with sepsis (F 4 M 3, mean age 35.1 years, range 30-55) were enrolled. Mean IL-18 serum level in AOSD patients [1298.7 pg/ml (range 0–6015)] was significantly higher from mean IL-18 serum level in sepsis [113.5 pg/ml (range 52–328), P=0.008]. ROC-AUC analysis of the serum concentration of IL-18 indicated that it was significantly diagnostic of AOSD, especially when active. The ROC-AUC analysis for the serum level of the IL-18 between patients with AOSD and sepsis was 0.712. At a cutoff point of 179 pg/ml, corresponding to the greatest sum of specificity and sensitivity, the specificity was 69.7% and the sensitivity was 87.5% for detection of AOSD (likelihood 2.89). When disease activity was scored according to Rau’s criteria, 21/30 (70%) patients were identified as active. These patients showed mean IL-18 serum levels (1793 pg/ml, range 0-6015) significantly higher than patient with “non active” disease (145.5 pg/ml, range 0-685, P=0.0039), as well as higher than sepsis (P=0.007). The ROC-AUC analysis for the serum level of the IL-18 between active patients with AOSD and sepsis was 0.845. At a cutoff point of 223 pg/ml, the specificity was 87.5% and the sensitivity was 80.9% for detection of AOSD (likelihood 6.48). Conclusions A significant difference in IL-18 serum levels between patients with AOSD and sepsis was found, especially in those patients with active disease. This evidence may confirm the potential utility of IL-18 in the differential diagnosis between AOSD and sepsis. Disclosure of Interest None Declared
ABSTRACT Background Although muscle pain is relatively frequent in primary Sjogren Syndrome (pSS)... more ABSTRACT Background Although muscle pain is relatively frequent in primary Sjogren Syndrome (pSS), a frank myositis is rare and reported only in 2.5-11% of patients. The most frequent symptoms are muscular weakness and myalgias and diagnosis is based on clinical, laboratory and histologic evaluation. Objectives To describe the prevalence and course of myositis in a multicenter cohort of patients with pSS. Methods Clinical and laboratory data from patients with a diagnosis of pSS were retrospectively collected. The clinical charts of patients with symptoms and/or signs of inflammatory myositis were carefully reviewed. Results One thousand one hundred seventy patients with pSS (52M, 1118 F, mean age 57.3 years; range 17–89 years) were considered. Mean age at diagnosis was 51±14 years and the mean follow up from diagnosis was 5.6 years (range 0–42 years). Ten female patients (0.85%) presented invalidating muscular weakness in the upper and lower limbs and/or severe myalgias. In 8/10 patients an increase of Creatine Phosphokinase (CK) serum level was present. A possible iatrogenic effect was excluded; in one case myositis started under statin therapy but didn’t disappear after drug discontinuation. In the 10 patients, mean age at pSS diagnosis was 47.4 years (range 14–68), while mean age of myositis diagnosis was 50.6 years (range 23–69). In six out of ten, muscular and sicca symptoms had occurred together at onset. Diagnosis of myositis was supported by clinical evidences and by the presence of increased CK serum levels. In 7/10 patients muscle biopsy was performed. In all biopsies signs of inflammation were present. In 1/7 patients (14.2%) the biopsy was typical of an inclusion body myositis, while in the remaining cases histological aspects of Dermatomyositis and Polymyositis (PM) were detected. Eight out of ten patients presented at least three criteria for inflammatory myopathy (according to Bohan and Peter criteria, 1975) suggesting a possible diagnosis of a true overlap syndrome. In one of these patients serum anti Jo-1 and anti RNP antibodies were detected. Six patients (60%) were successfully treated with DMARDs, while four (40%) did not respond. Thus, two were treated with IvIg, one showing complete remission of myositis, and the remaining two non-responder patients were treated with Rituximab, one showing complete remission. Conclusions Our retrospective study shows a lower prevalence of myositis in pSS than previously reported. Muscle involvement can occasionally represent the first symptom at disease onset. An overlap syndrome with inflammatory myopathies, above all PM, should be considered. Different therapeutic approaches can be used: DMARDs are effective in the majority of the patients, while IvIg and Rituximab may represent an available alternative therapeutic options in non-responder patients. Disclosure of Interest None Declared
Clinical and experimental rheumatology, Jan 19, 2015
In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequ... more In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was per...
To assess the quality of sexual life of women with primary Sjögren syndrome (pSS) and to identify... more To assess the quality of sexual life of women with primary Sjögren syndrome (pSS) and to identify its correlations with disease activity and damage, quality of life, and mood disorders. The quality of sexual life of 24 women with pSS was assessed with the Female Sexual Function Index (FSFI). Twenty-four healthy women, matched by age and hormonal status, were enrolled as controls. Mood disorders and quality of life were investigated using the Hospital Anxiety and Depression Scale (HADS) and the Medical Outcomes Study Short Form-36. Patients underwent a gynecological visit with vaginal pH measurement, cervicovaginal swabs, and Pap smears. Disease activity and damage were assessed by the European League Against Rheumatism Sjögren syndrome disease activity and damage indexes. Patients with pSS showed a pathological mean FSFI score (19.1 ± 7.33) significantly different from controls (p = 0.004), both in menstruating women (p = 0.006) and in menopausal women (p = 0.03). Major differences between the 2 groups were detected in dyspareunia (p < 0.005), lubrication (p = 0.006), desire (p = 0.004), and arousal (p = 0.018). The FSFI score was inversely correlated with age (p = 0.008) and anxiety HADS (p = 0.031). No early anatomical changes, swabs, and Pap smear alterations were revealed in patients with pSS; however, vaginal pH was higher than normal in premenopausal patients (6.0 ± 0.77). Both premenopausal and postmenopausal women with pSS have a worse sexual quality of life. We reported a greater prevalence of dyspareunia that is statistically significant when compared with controls. The FSFI could be a useful tool to assess this topic, but has been neglected in the care of patients with pSS heretofore.
Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life... more Fatigue and generalised pain are debilitating symptoms that negatively impact the quality of life in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS). Chronic widespread musculoskeletal pain and fatigue are the clinical hallmarks of fibromyalgia (FM), a clinical entity which can be associated to connective tissue disease. The aim of the present study was to assess the prevalence of FM syndrome, fatigue and widespread pain in SLE and pSS patients and to evaluate the contribution of inflammatory disease and FM on those constitutional symptoms. Fifty SLE and 50 pSS patients were enrolled in the study. Patients rated fatigue, pain, and disease activity using a 100-mm visual analogue scale and completed the Health Assessment Questionnaire and the Fibromyalgia Impact Questionnaire. Zung depression and anxiety scales were used to quantify mood disorders. Tender points were evaluated using an algometer. Disease activity score as evaluated for each SL...
To determine the clinical and laboratory differences between cryoglobulinaemic and hypergammaglob... more To determine the clinical and laboratory differences between cryoglobulinaemic and hypergammaglobulinaemic purpura in primary Sjögren's syndrome (pSS), in a large Italian multicentre cohort. Patients were selected according to the following criteria: fulfilling the American-European classification criteria for pSS, serum cryoglobulin and gammaglobulin levels evaluated, and lack of hepatitis C virus (HCV) infection. Multinomial analyses were performed by distinguishing three groups of pSS: (i) purpura associated with cryoglobulinaemic vasculitis (CV), (ii) purpura associated with hypergammaglobulinaemic vasculitis (HGV), and (iii) pSS patients without purpura (pSS controls). Patients with purpura but without cryoglobulins or hypergammaglobulinaemia were excluded. A total of 652 patients were enrolled in this study. Group 1/CV comprised 23/652 patients (3.53%), group 2/HGV 40/652 patients (6.13%), and group 3/pSS controls 589/652 (90.34%). The three groups were found to be signifi...
In primary Sjögren&am... more In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies. Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected. According to Bohan and Peter's criteria, inflammatory myopathy (IM) was suspected in case of muscular weakness associated with increased creatine-phosphokinase (CPK) or abnormal electromyography (EMG). When performed, muscle biopsies were analysed. In a cohort of 1320 patients, 17 (1.28%) presented muscular weakness [in some cases myalgias (7/17, 41.1%)], accompanied by increased CPK [13/17, (76.4%)] and/or abnormal EMG [13/14, (92.8%)]. Ten out of 17 (58.8%) fulfilled at least three diagnostic criteria for IM. Muscular biopsy was performed in 13/17 (76.4%) cases with histologically confirmed myositis in 6/13 (46.1%) (1"IBM-like"-5"PM-like"). In two "PM-like" cases, several fibres showed a decreased histochemical cytochrome C oxidase (COX) stain. Two biopsies tested…
To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS). An o... more To investigate pregnancy and fetal outcomes in patients with primary Sjögren syndrome (pSS). An obstetric history of 36 women with established diagnosis of pSS at pregnancy was obtained from a multicenter cohort of 1075 patients. In a subgroup case-control analysis, 12 deliveries in patients with pSS were compared with 96 control deliveries. Thirty-six women (31 with anti-SSA/Ro and/or anti-SSB/La antibodies) with an established diagnosis of pSS had 45 pregnancies with the delivery of 40 newborns. Two miscarriages, 2 fetal deaths, and 1 induced abortion were recorded. Mean age at the first pregnancy was 33.9 years; mean number of pregnancies was 1.25; 18/40 (45%) cesarean births were delivered; mean pregnancy length was 38.5 weeks (range 32-43), with 6 preterm deliveries. The mean Apgar score at 5 min was 8.9, mean birthweight was 2920 g (range 826-4060 g). Congenital heart block (CHB) occurred in 2/40 (5%) newborns. The reported rate of breastfeeding for at least 1 month was 60.5%. In 4/40 pregnancies (10%) a flare of disease activity was observed within a year from delivery. In the case-control subgroup analysis, 12 deliveries were compared with 96 controls and no significant differences were found. Patients with pSS can have successful pregnancies, which might be followed by a mild relapse. CHB was the only cause of death for offspring of mothers with pSS.
The differential diagnosis between rheumatic diseases and infectious conditions is a great challe... more The differential diagnosis between rheumatic diseases and infectious conditions is a great challenge in clinical practice. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory syndrome that shares several clinical and laboratory variables with sepsis. Interleukin (IL)-18 is overexpressed in AOSD, suggesting a possible role as a disease biomarker. The aim of our study was to detect IL-18 serum levels in a cohort of patients with AOSD and sepsis and to address its possible role as a biomarker for differential diagnosis. A group of unselected patients with AOSD diagnosed according to the Yamaguchi criteria and consecutive patients with sepsis diagnosed according to the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria were enrolled. The clinical and laboratory data were collected. In the AOSD group, disease activity was assessed by Pouchot's and Rau's criteria. IL-18 serum levels were detected by ELISA. Thirty-nine patients with AOSD and 18 patients with sepsis were enrolled. Two out of 18 patients with sepsis (11.1%) also fulfilled the Yamaguchi criteria. A significant difference was found in IL-18 serum levels between patients with active and inactive disease (p < 0.001), and it positively correlated with disease activity (p = 0.0003), ferritin serum level (p = 0.016), and erythrocyte sedimentation rate (p = 0.041). IL-18 was significantly increased in patients with AOSD when compared with sepsis (p = 0.014). For a cutoff of 148.9 pg/ml, this test had a specificity of 78.3% and a sensitivity of 88.6%. We have demonstrated that IL-18 can be a biomarker for differential diagnosis between AOSD and sepsis.
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