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Clinical Profiles of Males with SLE at their presentation in Korle -Bu Teaching Hospital

Clinical Profiles of Males with SLE at their presentation in Korle -Bu Teaching Hospital. Dr Huberta Quartey , Padiki Narh , Dr Ida Dey. Introduction. SLE is a clinically heterogenous autoimmune disease of unknown aetiology

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Clinical Profiles of Males with SLE at their presentation in Korle -Bu Teaching Hospital

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  1. Clinical Profiles of Males with SLE at their presentation in Korle-Bu Teaching Hospital Dr Huberta Quartey , PadikiNarh , Dr Ida Dey.

  2. Introduction • SLE is a clinically heterogenousautoimmune disease of unknown aetiology • It is characterized serologically by autoantibodies targeting self-proteins, notably ANAs, which are present in virtually all affected individuals. • SLE is said to be rare in Sub-Saharan Africa and even rarer in males. • The incidence of SLE is markedly increased in females of child-bearing age ratio being 8-15:1 • Pre-pubertal and post-menopausal ratios are much lower at 2–6:1 and 3–8:1 respectively

  3. The pathogenesis of SLE remains incompletely understood but most likely involves the interaction of genetic, hormonal and environmental factors • This striking predominance in females probably relates to the effect of endogenous sex hormones which have complex effects on the immune system • However, a full explanation for why the disease is so uncommon in men remains elusive. • Our study seeks to compare the clinical profiles of the males when the presented in the Korle-Bu teaching Hospital.

  4. Method • We retrospectively assessed the medical records of 8 of our male patients diagnosed with Systemic Lupus erythematosus(SLE). • 7 were attendant at the rheumatology clinic of Korle-Bu Teaching Hospital which has a patient population of about 200. • One of the patients was on the ward. • The patients history, examination and lab records were obtained • The laboratory tests that were compared were the Hemoglobin, ESR, urine Protein, Creatinine, Complements, C-Reactive protein, Antibody titre levels

  5. Results • The youngest was 17years • The oldest being 38yrs • The mean age being 29.5years • Males have been reported to experience first SLE-related symptoms at a mean age range of 26–38.4 years [9, 16] .which is comparable to our study

  6. Results • 62.% of the patients were diagnosed in less than a year from onset of symptoms • 25% of them diagnosed between 1 to 4years of onset of symptoms • All patients had general complaints on presentation • All patients had their musculoskeletal system affected with half being affected in the large joints and the other half being generalized

  7. Results • 75% had dermatological complaints • 62.5% had CNS complaints • 50% has respiratory complaints • 37.5% had cardiovascular complaints at presentation

  8. Results • 75% had an Hemoglobin level of <11g/dl at presentation • 25% had an HB between 11-18g/dl • 57.1% had an ESR greater than 50 • 42.9% had an ESR of between 1-20mmfall/hr • 66.7% had an elevated CRP • 33.3% had a normal CRP

  9. Results • 50% had ANA antibody titres of 1:320 • 16.7% : 1:640 • 33.3% : 1:160 • 5 patients had a positive ENA • 3 had yet done the test Anti-Ro(SSA)/Anti RNP • Positive in 80% • negative in 20% Antibody to ENA JO-1 • Negative in 4 patients

  10. Results Complement C3 • 57.1% - low • 47.9% - normal Complement C4 • 57.1%- normal • 47.9%- low Creatinine levels • 42.9% had normal creatinine levels • 42.9 had elevated levels • 14.9% had low levels

  11. Results Urine RE • Urine proteins 2+ -42.9% • 1+-28.9% • Normal-28.9% Urine AcR • Normal-33.3% • <500- 66.7% Urea • 57.1% -2.1-7.1 • 42.9%- >7.1 • One had done renal biopsy which showed a Stage V nephritis

  12. Results Albumin • <35g/dl-57.1% • 35-50g/dl-42.9%

  13. Discussion • The epidemiology of SLE in people of African origin clearly varies from one geographical setting to another. • It appears to be very rare in West Africa, increasing in frequency in Central and Southern Africa, • and of high frequency in America, the Caribbean and Europe.

  14. Discussion • From the study the mean age at presentation was 29.5years • Males have been reported to experience first SLE-related symptoms at a mean age range of 26–38.4 years (Murphy et al,2013) • From our study 62% had a less than a year from start of symptoms to diagnosis • The overall trend was to a shorter delay in diagnosis of male patients, with a range of 6–46 months between symptom onset and diagnosis in males (Murphy et al)

  15. Discussion • From our study musculoskeletal ,genitourinary and dermatological systems were the most affected at presentation. • According to Font et al(1992) patients of African American extraction frequently exhibit more significant renal disease, hypertension and discoid lupus and less photosensitivity • Discoid lesions and serositis more common compared with arthritis and malar rash less often

  16. Discussion • The LUpus in MInorities, NAture versus nurture (LUMINA) cohort is a well-known multiethnic US Cohort consisting of Hispanic, African American, and Caucasian patients • Of the 618 LUMINA patients enrolled at the time of the study, 555 were female, 63 male. Caucasians were overrepresented amongst males. • Musculoskeletal involvement was less frequent in males in this cohort(Schwartzman-Morris et al 2012)

  17. There was a lower incidence of musculoskeletal symptoms, alopecia and photosensitivity in men at diagnosis, with the suggestion of more prevalent serositis and discoid lupus. (Murphy et al) • Sthoeger et al observed a higher incidence of neurological disease, nephritis, thrombocytopenia, vasculitis and hepatosplenomegaly in male patients. • Tan et al (2012) found that men were more likely than women to have experienced end organ damage including neuropsychiatric, renal, cardiovascular, peripheral vascular disease, and myocardial infarction.

  18. Discussion Renal disease in SLE is a source of major morbidity and mortality; it develops in approximately 60% of patients with SLE, with a reported 5–22% of these patients progressing to end-stage renal disease requiring dialysis or transplant Studies have shown that lupus nephritis (LN) is more frequent in men than in women A recent study by Resende, et al. concluded that lupus nephritis may be more severe in men.Ingeneral, men may have more severe disease activity than women

  19. Discussion • Multiple factors including male sex, black race, presence of antiphospholipid antibodies, increased creatinine at the time of diagnosis, anemia, frequent nephritic flares, hypertension, and excessive prolonged proteinuria are all considered risk factors for increased progression to end-stage renal disease (ESRD) (Schwartzman Morris et al,2012) • From the study only one of the patients was able to do a renal biopsy which showed a stage V nephritis

  20. Conclusion • Overall experience with male patients with SLE is not extensive and the precise frequency of clinical and serological features differs from study to study

  21. References • 1.http://rheumatology.oxfordjournals.org/content/early/2013/05/02/rheumatology.ket160.full • https://www.hss.edu/conditions_ten-differences-male-female-lupus-patients.asp • http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1004835/ • Gender Differences in the Pathogenesis and Outcome of Lupus and of Lupus Nephritis • Julie Schwartzman-Morris1,2 and ChaimPutterman2 • http://www.ncbi.nlm.nih.gov/pubmed/22382348

  22. References • Effect of gender on clinical presentation in systemic lupus erythematosus • Grainne Murphy1 and David Isenberg

  23. Thank you

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