Abstract Objective Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France. Methods We ...collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature. Results We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18–68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8–21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1–117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p = 0.01). Conclusion Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome.
Sarcoidosis is a systemic disorder of unknown cause characterized by its pathological hallmark, the non-caseating granulomas, and by variable clinical course. While most of the cases affect people ...aged between 25 and 40 years, approximately 30 % of cases occur in older patients. Elderly-onset sarcoidosis (EOS) is defined as the onset of sarcoidosis in people over 65 years of age. Specific studies on the incidence and prevalence of sarcoidosis in this subgroup are scarce. Several studies suggest that the clinical features of EOS differ from those of sarcoidosis in younger patients. Compared with younger patients, fatigue, uveitis and specific skin lesions are more common, while erythema nodosum and chest x-ray abnormalities are less frequent. The diagnosis of EOS is challenging and may be delayed for many months because of its insidious onset, low prevalence and similarity to other more common disorders. When there is a granulomatous reaction in the elderly, clinicians should doubt the diagnosis and first think of tuberculosis, neoplasia or rare settings such as granulomatosis with polyangiitis or granulomatous reaction due to interferon and tumour necrosis factor-α (TNFα) blockers. A minor salivary gland biopsy also has a higher accuracy for diagnosis in the elderly. The current management of EOS remains empiric because of the lack of randomized, controlled studies. However, the approach to treatment is similar, regardless of the age of the patient. The treatment may be complicated by co-morbidities and increased risk of toxicities from usual treatments, particularly steroids. This review discusses the epidemiology, clinical course, prognosis and treatment of EOS.
To describe the characteristics, treatment, and outcome of patients with immune thrombocytopenia with clinical significance (ITPCS) associated with systemic lupus erythematosus (SLE).
This ...retrospective multicentre study included SLE patients who experienced ≥1 ITPCS (defined as ITP with attributable bleeding disorders and/or a platelet count <30 x109/L). Other causes of secondary thrombocytopenia were excluded. Major bleeding event (MBG) was defined as Khellaf score>8 and/or WHO score>2.
A total of 90 patients were included, the median (range) follow-up duration was 80 (6-446) months. ITP was diagnosed before SLE in 25 patients. They presented high rate of autoimmune haemolytic anaemia (15%), antiphospholipid antibody (62%), and antiphospholipid syndrome (19%). The 25 (28%) patients who experienced MBG had significantly more bleedings at ITP diagnosis and higher bleeding scores, and serositis and thrombosis during follow-up. They required significantly more treatment lines, transfusions, and hospitalizations. The 11 (12%) patients who experienced no bleeding event presented a significantly more restricted SLE phenotype (cutaneous and/or articular). Patients received a mean (range) of 4.2 (1-11) treatment lines. Corticosteroids and hydroxychloroquine allowed ITPCS overall response in one third of patients. The median relapse-free survival of rituximab (n = 34), azathioprine (n = 19), mycophenolate mofetil (n = 8), thrombopoietin-receptor agonists (n = 16), and splenectomy (n = 19) were 53, 31.5, 61, 24.5, and 78 months, respectively. Four patients experienced thrombotic events after splenectomy and one occurred under thrombopoietin-receptor agonist treatment.
SLE-ITCS patients displayed a high rate of haematological abnormalities and MBG patients exhibited higher morbidity. Management of thrombocytopenia was highly heterogeneous and many options seem viable.
Sarcoidosis is not rare in patients aged more than 65 years, but studies of elderly patients with sarcoidosis are scarce. We analyzed the characteristics and outcomes of patients in a French teaching ...hospital with late-onset sarcoidosis, defined as sarcoidosis diagnosed in patients aged 65 years or older, and compared them with those of younger patients with sarcoidosis. From 2002 to 2006, 30 patients were identified as having late-onset sarcoidosis and were compared to 70 patients randomly selected aged younger than 65 years. We compared clinical characteristics, laboratory data at diagnosis, severity, therapy, and outcome. The female to male ratio was higher in the late-onset sarcoidosis group than in the younger group (5:1 vs. 1:1, respectively; p = 0.003). Asthenia (30% vs. 10%; p = 0.012), uveitis (33.3 vs. 8.6%; p = 0.002), and specific skin lesions (36.7% vs. 15.7%; p = 0.002) occurred more frequently in patients with late-onset sarcoidosis than in younger patients. On the contrary, asymptomatic chest radiograph abnormalities (p = 0.031) and erythema nodosum (p = 0.016) were not reported in the group of elderly patients. The 2 groups were similar with regard to race, other organ systems involved, pulmonary function, radiographic stage, laboratory values, and severity. The proportion of patients with accessory salivary glands (p = 0.002) and skin (p = 0.023) biopsies was more often contributory to the diagnosis in the late-onset group.After a mean follow-up of 50 months, 1 death related to pulmonary mycetoma and 2 others unrelated to sarcoidosis occurred in the late-onset sarcoidosis group. The 5-year survival rate was 93.3% in the late-onset group compared with 100% in the young-onset group (p = 0.03), while overall mortality was not significantly different. The 2 groups were similar with regard to oral corticosteroid therapy and immunosuppressive use, although steroid-related adverse events were more common in the elderly group.In conclusion, we found certain clinical and diagnostic peculiarities in patients with late-onset sarcoidosis. Asthenia, uveitis, and specific cutaneous lesions were more frequent in this group, whereas erythema nodosum and disclosure on a routine chest roentgenogram were not observed. Biopsy of the minor salivary glands appears to be particularly pertinent for the diagnosis. Evolution and therapeutic management were not different in the 2 groups. However, the patients aged more than 65 years had more side effects related to the corticosteroid therapy.
To measure the association between SLE remission and scores of patients-reported outcome (PRO) measures.
We performed a prospective cohort study of SLE patients with a 2-year follow-up, using Lupus ...Patient-Reported Outcome (LupusPRO), Lupus Quality of Life (LupusQoL), Systemic Lupus Erythematosus Quality of Life (SLEQOL) and 36-item Short Form (SF-36) questionnaires. Remission was defined as remission off treatment (ROFT) and remission on treatment (RONT) according to the definitions of remission in SLE consensus. Mixed models accounting for repeated measures were used to compare groups as follow: ROFT and RONT vs no remission and lupus low disease activity state (LLDAS) vs no LLDAS.
A total of 1478 medical visits and 2547 PRO questionnaires were collected during the follow-up from the 336 recruited patients. A between-group difference in PRO scores reaching at least 5 points on a 0-100 scale was obtained in the following domains: lupus symptoms (LLDAS: +5 points on the 0-100 scale, RONT: +9, ROFT: +5), lupus medication (LLDAS: +5, RONT: +8, ROFT: +9), pain vitality (LLDAS: +6, RONT: +9, ROFT: +6) of LupusPRO; role emotional (LLDAS: +5, RONT: +8), role physical (RONT: +7 and ROFT: +7), bodily pain (RONT: +6), mental health (RONT: +5) and social functioning (RONT: +6) of SF-36. In contrast, a between-group difference reaching at least 5 points was not achieved for any of the LupusQoL and SLEQOL domains.
RONT, ROFT and LLDAS were associated with significant and clinically relevant higher QoL in most PRO domains of the LupusPRO (disease specific) and SF-36 (generic) questionnaires, but not with LupusQoL and SLEQOL disease-specific questionnaires.
Abstract
Objectives
Mediation analyses were conducted to measure the extent to which musculoskeletal (MSK) flares and depression affected physical health through excessive fatigue.
Methods
Mediation ...analyses were performed in a large multicentre cohort of SLE patients. Domains of the LupusQoL and SLEQOL questionnaires were selected as outcomes, MSK flares according to the SELENA-SLEDAI flare index (SFI-R) score and depression defined by Center for Epidemiologic Studies-Depression scale (CES-D) scale as exposures and different fatigue domains from MFI-20 and LupusQoL questionnaires as mediators. For each model, total, direct, indirect effects and proportion of effect mediated by fatigue (i.e. proportion of change in health-related quality of life) were determined.
Results
Of the 336 patients, 94 (28%) had MSK flares at inclusion and 99 (29.5%) were considered with depression. The proportion of the total effect of MSK flares on physical health impairment explained by fatigue ranged from 59.6% to 78% using the LupusQOL ‘Physical health’ domain and from 51.1% to 73.7% using the SLEQOL ‘Physical functioning’ domain, depending on the fatigue domain selected. The proportion of the total effect of depression on physical health impairment explained by fatigue ranged from 68.8% to 87.6% using the LupusQOL ‘Physical health’ domain and from 79.3% to 103.2% using the SLEQOL ‘Physical functioning’ domain, depending on the fatigue domain selected.
Conclusions
The effect of MSK flares and depression on physical health impairment is largely mediated by fatigue. Thus, the patient’s perception of disease activity as measured by physical health is largely influenced by fatigue. In addition, fatigue has a significant negative impact on quality of lifeof SLE patients with depression.
Trial registration
ClinicalTrials.gov, http://clinicaltrials.gov, NCT01904812.
Abstract Objectives To describe the characteristics, treatment and outcome of patients with immune thrombocytopenia with clinical significance (ITPCS) associated with SLE. Methods This retrospective ...multicentre study included SLE patients who experienced ≥1 ITPCS (defined as ITP with attributable bleeding disorders and/or a platelet count <30×109/l). Other causes of secondary thrombocytopenia were excluded. Major bleeding event (MBG) was defined as Khellaf score >8 and/or WHO score >2. Results A total of 90 patients were included, the median (range) follow-up duration was 80 (6–446) months. ITP was diagnosed before SLE in 25 patients. They presented a high rate of autoimmune haemolytic anaemia (15%), antiphospholipid antibody (62%) and antiphospholipid syndrome (19%). The 25 (28%) patients who experienced MBG had significantly more bleedings at ITP diagnosis and higher bleeding scores, and serositis and thrombosis during follow-up. They required significantly more treatment lines, transfusions and hospitalizations. The 11 (12%) patients who experienced no bleeding event presented a significantly more restricted SLE phenotype (cutaneous and/or articular). Patients received a mean (range) of 4.2 (1–11) treatment lines. Corticosteroids and HCQ allowed ITPCS overall response in one-third of patients. The median relapse-free survival of rituximab (n = 34), AZA (n = 19), MMF (n = 8), thrombopoietin-receptor agonists (n = 16) and splenectomy (n = 19) were 53, 31.5, 61, 24.5 and 78 months, respectively. Four patients experienced thrombotic events after splenectomy and one occurred under thrombopoietin-receptor agonist treatment. Conclusion SLE-ITCS patients displayed a high rate of haematological abnormalities and MBG patients exhibited higher morbidity. Management of thrombocytopenia was highly heterogeneous and many options seem viable.
OBJECTIVE:To describe characteristics, risk factors, and treatment outcome of progressive multifocal leukoencephalopathy (PML) complicating sarcoidosis.
METHODS:A retrospective chart and literature ...review was performed. Patients were identified through records from physicians of the Groupe Sarcoïdose Francophone. Each case was compared with 3 controls.
RESULTS:Ten cases were found (8 men). The median age at sarcoidosis diagnosis was 34.9 (±6) years. PML and sarcoidosis were diagnosed concomitantly in 2 cases, while sarcoidosis was previously known in 8 cases, including 7 cases treated with steroids (mean time between sarcoidosis diagnosis and PML was 114 ±99 months). The mean CD4 cell count was 215 (±139)/mm. Neurosarcoidosis was thought to be the problem in 8 cases and treatment was intensified, delaying PML diagnosis by 4.5 (±3.9) months. Eight patients received PML-specific treatment. On the whole, 6 patients died of PML within a mean time of 8 (±4.3) months. Patients with PML were significantly younger than controls. When combining our 10 patients with another 20 from the literature, we found that 17 patients (57%) died from sarcoidosis-associated PML; thus, the fatality rate was 57%.
CONCLUSIONS:PML during sarcoidosis is often misdiagnosed. It is not associated with severe CD4 lymphocytopenia. Fatality rate is high in comparison with PML associated with other conditions. Interrupting immunosuppression remains the mainstay of treatment.
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