Familial juvenile systemic lupus erythematosus in Arab children

Sulaiman Al-Mayouf, Reem Abdwani, Safia Al-Brawi

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Aim of this study is to analyze the demographic, clinical, and biochemical features, and survival of familial juvenile systemic lupus erythematosus (FJSLE) in Arab children. The medical records of children with FJSLE seen at three pediatric rheumatology clinics in Saudi Arabia and Oman were retrospectively reviewed. All included children have met the following criteria: Arab ethnicity, definite diagnosis of SLE using the revised 1982 American College of Rheumatology classification criteria and family history of more than one affected sibling with SLE. The collected data included: Gender, age at diagnosis, clinical and laboratory features at diagnosis. Unusual co-morbidity and mortality associated with the disease were studied. There were 50 children with FJSLE belonging to 18 families; the frequency of FJSLE in our cohort was 20.8%. The mean age at onset of SLE was 86 months (range, 18-168 months), while the mean age at diagnosis was 95 months (range, 24-192 months), and the mean duration of follow-up was 60.9 months (range, 7-132 months). The proportion of girls was predominant (78%). Autosomal recessive mode of inheritance was strongly suggested in number of our families. Mucocutaneous manifestations, arthritis, and nephritis were the most frequent features. Thirty-five patients had renal lesions, 18 of them had class IV nephritis according WHO classification. All patients were treated with different doses of steroid and immunosuppressive drugs; 37 (74%) patients received cyclophosphamide, and 6 patients treated with Rituximab. There were 5 patients required dialysis due to ESRD and 8 deaths related to SLE during the period of follow-up. FJSLE is not uncommon in our society. These findings may be helpful in identifying SLE patients with a stronger genetic predisposition; hopefully, one or more additional risk loci can be identified in multiplex Arab families that are different from what has been reported in other ethnic populations.

Original languageEnglish
Pages (from-to)1939-1943
Number of pages5
JournalRheumatology International
Volume32
Issue number7
DOIs
Publication statusPublished - Jul 2012

Fingerprint

Systemic Lupus Erythematosus
Nephritis
Oman
Saudi Arabia
Clinical Laboratory Techniques
Rheumatology
Genetic Predisposition to Disease
Immunosuppressive Agents
Age of Onset
Cyclophosphamide
Chronic Kidney Failure
Arthritis
Medical Records
Siblings
Dialysis
Steroids
Demography
Pediatrics
Morbidity
Kidney

Keywords

  • C1q deficiency
  • Familial lupus
  • Juvenile systemic lupus erythematosus
  • Mode of inheritance
  • Multiplex lupus families

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

Familial juvenile systemic lupus erythematosus in Arab children. / Al-Mayouf, Sulaiman; Abdwani, Reem; Al-Brawi, Safia.

In: Rheumatology International, Vol. 32, No. 7, 07.2012, p. 1939-1943.

Research output: Contribution to journalArticle

Al-Mayouf, Sulaiman ; Abdwani, Reem ; Al-Brawi, Safia. / Familial juvenile systemic lupus erythematosus in Arab children. In: Rheumatology International. 2012 ; Vol. 32, No. 7. pp. 1939-1943.
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abstract = "Aim of this study is to analyze the demographic, clinical, and biochemical features, and survival of familial juvenile systemic lupus erythematosus (FJSLE) in Arab children. The medical records of children with FJSLE seen at three pediatric rheumatology clinics in Saudi Arabia and Oman were retrospectively reviewed. All included children have met the following criteria: Arab ethnicity, definite diagnosis of SLE using the revised 1982 American College of Rheumatology classification criteria and family history of more than one affected sibling with SLE. The collected data included: Gender, age at diagnosis, clinical and laboratory features at diagnosis. Unusual co-morbidity and mortality associated with the disease were studied. There were 50 children with FJSLE belonging to 18 families; the frequency of FJSLE in our cohort was 20.8{\%}. The mean age at onset of SLE was 86 months (range, 18-168 months), while the mean age at diagnosis was 95 months (range, 24-192 months), and the mean duration of follow-up was 60.9 months (range, 7-132 months). The proportion of girls was predominant (78{\%}). Autosomal recessive mode of inheritance was strongly suggested in number of our families. Mucocutaneous manifestations, arthritis, and nephritis were the most frequent features. Thirty-five patients had renal lesions, 18 of them had class IV nephritis according WHO classification. All patients were treated with different doses of steroid and immunosuppressive drugs; 37 (74{\%}) patients received cyclophosphamide, and 6 patients treated with Rituximab. There were 5 patients required dialysis due to ESRD and 8 deaths related to SLE during the period of follow-up. FJSLE is not uncommon in our society. These findings may be helpful in identifying SLE patients with a stronger genetic predisposition; hopefully, one or more additional risk loci can be identified in multiplex Arab families that are different from what has been reported in other ethnic populations.",
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