Rita Fonseca1, Francisca Aguiar1, Mariana Rodrigues2,
Iva Brito1, 3
1. Serviço de Reumatologia, Centro Hospitalar de São João, Porto, Portugal
2. Serviço de Pediatria, Centro Hospitalar de São João, Porto, Portugal, 3Faculdade de Medicina da Universidade do Porto, Porto, Portugal
Background: Neuropsychiatric involvement in syste- mic lupus erythematosus (NPSLE) includes the neuro- logical syndromes of the central, peripheral or autono- mic nervous systems and psychiatric disorders. Some studies suggests that juvenile systemic lupus erythe- matosus (jSLE) has a more serious course and a more frequent neuropsychiatric involvement than adult on- set, coursing with significant morbidity and mortality. Its prevalence ranges from 22 to 50.9%.
Objectives: To assess neuropsychiatric manifestations in juvenile-onset SLE patients and study the predictors and association to disease characteristics.
1020 30 40 50Age
JSA min [mm2]
Discrimination based in JSA.Age60 70 80 90 10015013011090705030CASEDevidinglineCONTROLSNcase=152Ncontrol=50Odds R=9,8Accuracy=75,7%Sensitivity=76,0%Specificity=75,6%0,120 30 40 50AgeJSW min [mm2]Discrimination based in JSW.Age60 70 80 90 1008,17,16,15,14,13,12,11,1CASEDevidinglineCONTROLSNcase=152Ncontrol=50Odds R=15,6Accuracy=79,7%Sensitivity=80,0%Specificity=79,6%
10 20 30 40 50 Age JS A m in [m m 2]
Discrimination based in JSA.Age
60 70 80 90 100 150 130 110 90 70 50 30 CASE Deviding line CONTROLS Ncase=152 Ncontrol=50 Odds R=9,8 Accuracy=75,7% Sensitivity=76,0% Specificity=75,6% 0,1 20 30 40 50 Age JS W m in [m m 2]
Discrimination based in JSW.Age
60 70 80 90 100 8,1 7,1 6,1 5,1 4,1 3,1 2,1 1,1 CASE Deviding line CONTROLS Ncase=152 Ncontrol=50 Odds R=15,6 Accuracy=79,7% Sensitivity=80,0% Specificity=79,6%
Methods: Retrospective observational study was per- formed including consecutive patients with jSLE (di- sease onset before 16 years of age,) that fulfilled Ameri- can College of Rheumatology (ACR) criteria followed in our Paediatric Rheumatology Unit. Clinical, demo- graphic and laboratory characteristics were retrospecti- vely collected by consulting the medical records. The neuropsychiatric manifestations were defined according to the nomenclature and classification of ACR. Patients with NPSLE were compared with others using Student t-test, Mann-Whitney test, Chi-square or Fi sher test. (SPSS 23.0). Significance level was set as <0.05. Results: 38 patients were included, 92.1% (35) were female, with a mean age at diagnosis of 12.9 ± 3 years. Median period between onset of symptoms and dia - gnosis of SLE was 0.3 [0-1.2] years and median dura- tion of follow-up was 14 [0.75-26].
Neuropsychiatric manifestations of the SLE were obser ved in 8 (21%) patients: chorea (n=1), psychosis (n=2), seizures (n=1), transverse myelitis (1), stroke and weakness (n=1), headache (n=2).
Among the 8 patients with NPSLE, 7 were female and 1 was male. The median time between diagnosis and NPSLE manifestation was 1.75 [0-3] years. The two patients with headaches were treated with oral glu- cocorticoid. The remaining patients were treated me - thylprednisolone pulses, 3 were treated with cyclo - phosphamide and 1 with rituximab.
Full recovery was observed in almost patients, ex- cept for those with headaches that had many recur- rences of symptoms during follow up.
Comparing patients that had NPSLE with the re- maining, we found a statistically significant higher SLEDAI and SLICC scores in those with NPSLE (4 vs 2, p=00.04 and 1.5 vs 0, p=0.003, respectively).The age, sex and disease duration were comparable between the groups.
NPSLE was associated with more frequent lupus nephritis (75% vs 43.3%, p=0.03) and presence of an- tiphospholipide syndrome (APS) (20% vs 11.2%, p=0.04).
NPSLE patients also had higher lupus anti-coagu- lant prevalence (25% vs 10.7%) and anticardiolipine antibodies (23.3% vs 12.5%) %), yet none of the abo- ve reached statistical significance.
Conclusions: The neuropsychiatric manifestations are frequently observed in patients with jSLE and central nervous system manifestations were more frequently observed than manifestations affecting the peripheral nervous system. Our study suggests an association be -
tween NPSLE and lupus nephritis, higher disease acti- vity and the presence of APS. We observed a good outcome with complete resolution in the majority of the cases.
P94 – INFLAMMATORY THORACIC BACk PAIN IN A PATIENT WITH PSORIASIS AS THE INITIAL PRESENTATION OF A TAkAYASY ARTERITIS
Vítor Teixeira1, Carlos Miranda Rosa1,
Mariana Roque2, Filipa Oliveira Ramos1,
Elsa Vieira-Sousa1, José Carlos Romeu1
1. Rheumatology Department, Hospital de Santa Maria (CHLN), Lisbon Medical and Academic Centre, Lisboa, Portugal
2. Imagiology Department, Hospital de Santa Maria (CHLN), Lisbon Medical and Academic Centre, Lisboa, Portugal
Introduction: Takayasu Arteritis (TA) is a rare granu- lomatous vasculitis affecting large vessels, mainly the aorta (Ao) and its branches, usually occurring in young women (Jennette JC, 2013). It may present with a broad spectrum of symptoms, but inflammatory tho- racic back pain is uncommon at the time of diagnosis (Kerr GS, 1994).
Clinical Case: A 30 year old woman with psoriasis was referred due to a 6 months upper back pain, without limb radiation, more intense at night and associated with 30 minutes morning stiffness. NSAIDs initially re- lieved the pain but it got worst throughout the months with loss of response. She also referred vespertine low grade fever in the 2 months previous to admission. She
FIGURE 1.Thoracic CT, axial view - thickening of the walls of the thoracic aorta (arrow) and pulmonary trunk and its branches (star)
had been diagnosed by a non-rheumatologist with pso- riatic spondyloarthritis, despite a normal spinal tomo- graphy (CT) and magnetic resonance. There was no li- mitation in the amplitude of movements of the spine, no pain was elicited by pressure over spinous process but by pressure over the paravertebral muscles of the upper back. Sacroiliitis manoeuvres were negative and there was no evidence of peripheral arthritis or enthe- sitis. She had normal and equivalent blood pressure and pulses, measured in both upper and lower limbs. Blood tests revealed a low Hb 10.1g/dL, neutrophilia 9.01x10^9/L, thrombocytosis (637x10^9/L), elevated ESR (94mm/h) and CRP (10.9mg/dL); elevated alkali- ne phosphatase 329U/L and GGT 87U/L but normal bilirubin and transaminases. Antibodies screening (ANAs, ANCAs, LKM, AMA, ASMA, AAF, RF and anti- -CCP), blood cultures, serologic markers for infectious agents, mantoux test and IGRA were negative.
Due to suspicion of referred pain it was requested a thoracic CT. A thickening of the wall of the thoracic Ao, pulmonary trunk (PT) and its right and left bran- ches was visualized. A angio-CT showed a decrease in the diameter of the left primitive carotid artery (CA) with concentric thickening of the wall and ectasia in the origin of the left internal CA. Left external CA also presented a decreased diameter; left internal CA had coiling. PET-Scan confirmed vasculitis of thoracic Ao, left CA and distal PT. Doppler US scan also showed axillary and brachial arteries with marked diffuse thi - ckening of the arterial walls and “halo sign”. Abdomi- nal US revealed a homogenous hepatomegaly with nor- mal contours and biliary ducts. The diagnosis of TA was established and the patient was initially treated with prednisolone 1mg/kg/day, aspirin 100 mg/day and methotrexate 15 mg/week, with improvement of sym - ptoms, acute phase reactants (AFR) and normalization of hepatic enzymes.
Discussion: Careful assessment of back pain is funda- mental to disclose less common but potentially severe diseases, including non-vertebral conditions such as TA. The characteristics of the back pain of our patient could have been easily attributed to axial spondy- loarthritis. However, the absence of clinical signs and the normality of the imaging study of the spine exclu- ded this diagnosis. Hepatic involvement in TA, obser- ved in this case, has not been appropriately studied but as been previously documented (Durant C, 2011).
This report emphasizes that persistent back pain accom panied by systemic manifestations and elevation of AFR requires the consideration of extra-vertebral di-
sorders, including, in the case of the young woman, initial inflammatory phase of the TA.
P85 – HIP INVOLVEMENT IN