G. La Montagna

Severe polymyositis due to Toxoplasma gondii in an adult immunocompetent patient: a case report and review of the literature.

Toxoplasmosis, a worldwide zoonosis caused by a coccidian parasite Toxoplasma gondii, is more often asymptomatic in immunocompetent patients. We report the case of a 38-year-old immunocompetent male with a polymyositis as the presenting manifestation of T. gondii infection. The patient was hospitalized for a 30-day history of fever (T max 39.5°C), muscle pain, and progressive weakness of the muscles. A diagnosis of polymyositis was made, and he was started on corticosteroid treatment, which caused no reduction of symptoms. After finding a positive polymerase chain reaction (PCR) assay for T. gondii, together with additional clinical findings, a diagnosis of acute toxoplasmosis was made. Specific treatment with pyrimethamine and sulfadiazine was started, with a progressive reduction of symptoms and normalization of laboratory tests.

PREVALENCE AND INCIDENCE OF OSTEOPOROTIC FRACTURES IN PATIENTS ON LONG-TERM GLUCOCORTICOID TREATMENT FOR RHEUMATIC DISEASES: The Glucocorticoid Induced Osteoporosis Tool, GIOTTO STUDY.

Osteoporosis and fractures are common and invalidating consequences of chronic glucorticoid (GC) treatment. Reliable information regarding the epidemiology of GC induced osteoporosis (GIOP) comes exclusively from the placebo group of randomized clinical trials while observational studies are generally lacking data on the real prevalence of vertebral fractures, GC dosage and primary diagnosis. The objective of this study was to evaluate the prevalence and incidence of osteoporotic fractures and to identify their major determinants (primary disease, GC dosage, bone mineral density, risk factors, specific treatment for GIOP) in a large cohort of consecutive patients aged >21 years, on chronic treatment with GC (≥5 mg prednisone - PN - equivalent) and attending rheumatology centers located all over Italy. Glucocorticoid Induced OsTeoporosis TOol (GIOTTO) is a national multicenter cross-sectional and longitudinal observational study. 553 patients suffering from Rheumatoid Arthritis (RA), Polymyalgia Rheumatica (PMR) and Connective Tissue Diseases (CTDs) and in chronic treatment with GCs were enrolled. Osteoporotic BMD values (T score <-2.5) were observed in 28%, 38% and 35% of patients with CTDs, PMR or RA at the lumbar spine, and in 18%, 29% and 26% at the femoral neck, respectively. Before GC treatment, prevalent clinical fractures were reported by 12%, 37% and 17% of patients with CTDs, PMR, or RA, respectively. New clinical fragility fractures during GC treatment were reported by 12%, 10% and 23% of CTDs, PMR and RA patients, respectively. Vertebral fractures were the prevailing type of fragility fracture. More than 30% of patients had recurrence of fracture. An average of 80% of patients were in supplementation with calcium and/or vitamin D during treatment with GCs. Respectively, 64%, 80%, and 72% of the CTDs, PMR and RA patients were on pharmacological treatment for GIOP, almost exclusively with bisphosphonates. The GIOTTO study might provide relevant contributions to clinical practice, in particular by highlighting and quantifying in real life the prevalence of GIOP and relative fractures, the frequency of the main risk factors, and the currently sub-optimal prevention. Moreover, these results emphasize the importance of the underlying rheumatic disease on the risk of GIOP associated fractures.

The predictive value of attributes of pain to classify rheumatoid arthritis.

Abstract: Out of 2300 patients with rheumatic diseases 1627 were analysed to develop a classification of rheumatoid arthritis based on clinical attributes of pain. Of these, 641 patients had the disease and 986 were controls with other rheumatic conditions. For traditional format classification, six of eight variables were selected: pain at a fixed joint; symmetrical pain; continuous pain; pain mainly present at night or in the morning; pain following joint pressure; and pain decreased by load/movement. The occurrence of four or more of these features was associated with a 72.1% sensitivity and a 79.1% specificity. A classification tree constructed on four features that showed the greatest diagnostic power (symmetrical pain, pain mainly present at night or in the morning, pain at joint pressure, continuous pain), was associated with a 75.8% sensitivity and a 77.0% specificity.

AB0498 Low Dose Pulse IV Methylprednisolone as Induction Therapy for Lupus Nephritis: A Preliminary Study

Background Glucocorticoids (GC) pulses (750mg/day for 3 consecutive days) represent the induction arm of lupus nephritis trial (1) and are commonly used in clinical practice. This regimen can be complicated by adverse events as recently stressed by a EULAR Consensus Committee (2). Current knowledge showed that pulses IV methylprednisolone (250 mg/day) are known to both saturate glucocorticoid receptors and assure a complete non genomic effect (3). Objectives To evaluate the effectiveness and safety of 250 mg IV methylprednisolone pulses in combination with immunosuppressive therapy in the treatment of lupus nephritis. Methods Twelve SLE patients (female 89%) aged from 21 to 70 years (median 33.5) and with disease duration ranging from 0.5 to 24 years (median 9.5), all of whom satisfied 2012 ACR criteria (4) for the classification of SLE, were evaluated in the study. All of them had active nephritis based on proteinuria >0.5 g/24h and active urinary sediment or biopsy and 7 patients had impairment of renal function (i.e.,serum creatinine >1.2 mg/dl). All were treated between 2012 to 2013 by EURO-Lupus nephritis regimen modified only in the range of 3 daily pulses of 250 mg IV methylprednisolone (instead of 750mg). Clinical response was evaluated at 3 and 6 months according to response criteria indicated in Euro-lupus nephritis trial (1) and the recently proposed outcome measures for lupus nephritis (5). Adverse events occurring from study inclusion to the last follow up visit were recorded. Results At enrollement patients presented an ECLAM score ranging from 1 to 10 (median 7), a SLEDAI score ranging from 3 to 18 (median 12); 24 hour proteinuria ranging from 0.675 to 14 g (median 2.86 g), serum creatinine ranging from 0.5 to 2.1 mg/dl (median 1 mg/dl). Three patients underwent a renal biopsy that pointed out a proliferative lupus glomerulonephritis (2 patients WHO class III-1 patient WHO class V). The table shows the changes detected at 3 and 6 months. Timing ECLAM/SLEDAI (Δ%) Complete response** Partial response** No response** Treatment failure* 3 months −65.7/−68.1 5/12 (41.7%) 3/12 (25%) 4/12 (33%) 4/12 (34%) 6 months −74.0/−92.4 7/10 (70%) 2/10 (20%) 1/10 (10%) 2/10 (25%) * (1); ** (5). No adverse events were registered. Conclusions Preliminary data of this ongoing study seem to indicate that pulse methylprednisolone (250mg/day) might be as effective as the used 750mg dosage as induction therapy for lupus nephritis. References Houssiau F A, et al. Arthritis Rheum 2002, 46: 2121–31 Duru N, et al. Ann Rheum Dis 2013;72:1905-13. Buttgereit F, et al. Arthritis Rheum 2004. 50: 3408–17 Petri M, et al. Arthritis Rheum 2012;64:2677-86. Wofsy D, et al. Arthritis Rheum 2013 65: 1586–91 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4227

THU0395 Adult Onset Still's Disease: A Multicenter Retrospective Cohort Study of 233 Italian Patients

Background Adult onset Stills disease (AOSD) is a rare rheumatic disease with an estimated prevalence of less than 1 case per 100,000 population. The diagnosis is difficult and is often delayed due to the lack of specific diagnostic tests and the need to rule out other pathological entities. Objectives To describe the clinical characteristics of a multicenter Italian case series of patients with AOSD. Methods 14 Italian University Hospital centers participated in the study. A standardized medical record containing clinical data, laboratory investigations, disease patterns and the different therapies has been sent to all participating centers. Each center collected data retrospectively. Results In the first wave of data collection, from March to May 2013, 233 patients were included with AOSD according to Yamaguchi criteria (125 males, 53.6%, mean age at onset: 40.2±16.2 years, time between onset and diagnosis: 242±650 days). The most frequent manifestations at onset were: arthralgia (93.0%), fever (92.6%), leukocytosis (89.0%), typical rash (67.7%), sore throat (61.8%), lymphadenopathy/splenomegaly (60.4%), increased liver enzymes (53.5%). In 56.4% of patients ferritin was increased more than 5 times the normal threshold. Fever intensity was recorded up to 41 ° C (mean 39.1±0.7), with a single daily peak in 27.3% of cases and 2 peaks in 51.9% of cases. The average duration of febrile episodes was 10.3 days with variable interval between episodes (average 3.2 days). Based on clinical evolution the disease was classified polycyclic systemic in 40.8%, chronic articular polycyclicin 30.7%, systemic monocyclic in 23.9% and chronic articular monocyclic in 4.6%. In 21.9% of cases, corticosteroids and traditional DMARDs have not been able to control the disease while biological drugs have been shown to be effective. 56 cycles of biological drugs were used in 51 patients: anakinra in 22 cases, etanercept in 15 cases, infliximab in 8 cases, adalimumab in 7 cases, rituximab in 3 cases, tocilizumab in 2 cases, abatacept and golimumab in 1 case each (anti- TNFalpha 52.5%, 37.3% anakinra, other 10.2%). Conclusions This study presents the largest Italian multicentre series of AOSD patients. The main clinical and laboratory characteristics are in line with those reported in the literature. In more than 20% of patients biologics are the only drugs that allow to control the disease. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.5300

THU0412 Prevalence and Incidence of Osteoporotic Fractures in Patients on Long-Term Glucocorticoid Treatment for Rheumatic Diseases: The Glucocorticoid Induced Osteoporosis Tool, Giotto Study.

Background Osteoporosis and fractures are common and invalidating consequences of chronic glucorticoid (GC) treatment. Reliable information regarding the epidemiology of GC induced osteoporosis (GIOP) are coming exclusively from the placebo group of randomized clinical trials while observational studies are generally lacking data on the real prevalence of vertebral fractures, GC dose and primary diagnosis. Objectives The objective of this study was to evaluate the incidence of osteoporotic fractures and to identify their major determinants (primary disease, GC dose, BMD values, specific treatment for GIOP, etc.) in a large cohort of consecutive patients aged > 21 years, on chronic treatment with GC (≥5 mg prednisone –PN- equivalent), attending rheumatology centres located all over Italy. Methods Here the results of an interim analysis of the first 553 enrolled patients are presented. Systemic Lupus Erythematosus or other Connectivites (CON), Polymialgia Rheumatica (PMR), and Rheumatoid arthritis (RA) were the underlying diseases. Results In the table are shown the main clinical characteristics : Conclusions In real life the prevalence of clinical fractures in patients with rheumatic diseases is high even before GC treatment and the incidence of new clinical fractures while on chronic GC treatment (PN- equivalent ≥ 5 mg/day) is somewhat lower than hitherto assumed. These results emphasize the importance of the underlying disease in the risk of GIOP associated fractures. Disclosure of Interest None Declared