Background Systemic lupus erythematosus (SLE) is a multisystem disease affecting almost all of the organ systems. Children manifests frequently with signs and symptoms such as: fever, fatigue, arthralgia or arthritis, and rash. Renal involvement occurs 40-70% of all SLE patients and is a major cause of morbidity and hospital admissions. Male lupus represent only a small percentage because if its female preponderance, but it may not be appropriate that these cases should be underestimated to females with SLE in terms of most health-related issues. Lupus is a lifelong illness and complications may arise if no prompt treatment be instituted.
Objectives To describe clinical, laboratory profile and outcome of Filipino Male Lupus patients seen at the PGH from 2004-2013
Methods All pediatric patients who fulfilled at least 4 of the 1997 revised criteria of the American College of Rheumatology or the 2012 New SLICC Classification Criteria for Systemic Lupus Erythematosus that were seen at PGH over a 10-year period, male population were selected. Excluded in the study were drug-induced lupus, neonatal lupus, discoid lupus or the mixed connective tissue disease. Clinical presenting features, clinical manifestation during the illness and laboratory findings during the course of the disease for each patient were collected from the Pediatric Rheumatology section. Hospital Charts available at the Medical Records Section were also retrieved and reviewed.
Results A total of 215 pediatric patients diagnosed with SLE were seen. The female to male ratio was 6:1 (186 females, 29 males).Male lupus represents 13% of the total SLE patients. Thirteen out of the 29 charts were retrieved. The overall mean age at diagnosis of the male cases was 13.3 years (SD 3.4) ranging from 6 years to 18 years old. Among the 13 patients reviewed, edema (53.8%), fever (46.1%), abdominal pain (38.4%) and easy fatigability (30.7%) were the most common features at disease onset. At the time of diagnosis, the most common manifestations were renal involvement (84.6%), malar rash (53.8%) and oral ulceration (46.1%). All of 9 male lupus patients who had ANA titers determination had a 100% positive ANA results. Three patients who had Anti-dsDNA antibodies tested had positive results. Low complement values were seen in 83.3%. The follow-up period ranged from 0.2-2 years with a mean duration of 1.2±0.6 years. Four went into remission and is still being seen at the Rheumatology Clinic;3 patients died, 3 were transitioned to adult rheumatology section and 3 were lost to follow-up. The causes of death include disseminated intravascular coagulopathy, septic shock and active lupus.
Conclusions Edema was the most common feature at the onset of the disease in male lupus Filipino children. At the time of diagnosis, the most common manifestations were renal involvement (84.6%), malar rash (53.8%) and oral ulceration (46.1%). The frequency of renal involvement was relatively high compared to other studies. Male pediatric lupus patients had positive ANA and anti-dsDNA and low C3 results. Causes of death noted were:1 male patient suffered from an active disease, another patient manifested septic symptoms and third case died from disseminated intravascular coagulopathy. Early recognition and diagnosis will lead to prompt institution of treatment that will benefit the SLE population particularly the male pediatric age group.
Disclosure of Interest None declared
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