Background JSLE is a multisystemic auto immune disease but its onset not always is multiorganic. Different types of onset have been recognized. Multysistemic onset is the typical type, an oligosystemic (less than 4 target organs involved) is also observed, other patients have an atypical onset of SLE and others had an anti-phospholipid syndrome as a main manifestation. The diagnosis may be delayed if the patient does not fulfill the ACR criteria’s and it can be associated with poor prognosis. The course of the disease is not necessarily related to the type of debut. Different classifications have been used to describe the disease course. The organ damage is established using Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI).
Objectives Describe the type of debut of a group of patients with jSLE and determine its relationship with the clinical course in three medical centers in Bogota, Colombia.
Methods It is a descriptive bidirectional multicenter study. All patients hadconfirmed diagnosis of jSLE by ACR’s criteria. The Hopkins Lupus Cohort study classification was used to describe SLE patterns of disease activity: relapse remitting (RR), chronic activity (CA) and long quiescence (LQ). We related the type of onset and course of the disease. Damage was established using SDI. The minimum follow up time were 6 months and complete medical record had to be available.
Results This seriesincluded 66 jSLE patients 81.8% were female. The mean age of illness onset was 11.4 years (7-15).The mean follow-up time was44.8 months (6-108).35/66 had an oligosystemic onset (53%), 28 (42.4%) were multisystemic, 2/66 (3%) had antiphospholipid syndrome at the onset and 1/66 (1.5%) had an atypical debut.The three most frequent clinical manifestations at onset of jSLE were arthritis (62.1%), cytopenias (60.6%) and renalinvolvement (51.5%). 52, 9% of patients had type IV lupus nephritis. 4.5% patients were antinuclear antibody negative. The most common type of course was RR (69,7%). By relating the debut type and clinical course was observed that 71.4% of oligosystemic patients developed a RR pattern course, which was equal at the multisystemic onset. However, CC lupus pattern was more frequent in patients with multisystemic onset (17, 9% vs 8,6%) but not statistically significant (p 0,074). The frequency of organ damage at the last consultation was 43.9%. The most common type of damage was growth failure (75, 8%). There was increased frequency of organ damage in patients with multisystem debut when compared with other groups (p 0,054).
Conclusions jSLE debuthave high frequency of oligosystemic organ involvement. The most frequent type of course was RR. The multisystemic onset group showed a tendency to develop a chronic activity course. Multisystemic debut has a higher frequency of organic damage which negatively impacts the prognosis of those patients.
Taddio A et al. Prognostic impact of atypical presentation in pediatric systemic lupus. J of Pediatrics 2010; 156: 972-977
Otten MH et al. Disease activity patterns in juvenileSLE and its relation to early aggressive treatment. Lupus 2010; 19: 1550-1556
Disclosure of Interest None Declared