CLINICAL AND AUTOANTIBODY PROFILES IN MALES AND FEMALES PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A RESTROSPECTIVE STUDY IN 603 BRAZILIAN PATIENTS

Seventy-nine cases of autoimmune thrombocytopenia seen by the Baylor Hematology section of The Methodist Hospital between 1991 and 1996 were retrospectively reviewed to assess the effectiveness of danazol in the treatment of autoimmune thrombocytopenia. Among the 42 patients who received danazol, the mean initial platelet count prior to treatment was 24.3 ± 17.4 (SD) × 109/L with a mean duration of disease of 53 months. Most cases were idiopathic, but some patients had underlying secondary disorders (rheumatoid arthritis, systemic lupus erythematosus, HIV, and/or Evans' syndrome). Overall 57% of the patients treated with danazol had an excellent or a good response with three patients who had unmaintained remission for >11 months. Minimal side effects were noted. Fifty percent of the patients with associated secondary disorders achieved an excellent or good response. The hemolytic component of all three Evans' syndrome cases was well controlled with danazol. In two cases, danazol was effective where a variety of other treatment regimens were not. An excellent or a good response was found in 58%, 62%, and 53% in patients >65 years old, between 45 and 65 years old, and <45 years old, respectively. Response rates were similar in males and females, Seventy percent of the nonsplenectomized patients had an excellent or a good response compared to 33% in postsplenectomy patients. Overall in view of its favorable side-effect profile, it is rational early on to attempt to abrogate the need for splenectomy, it may salvage splenectomy failures, and there is a reasonable response rate in those refractory to multiple prior therapies. Key Words: Autoimmune thrombocytopenia—Idiopathic thrombocytopenic purpura—Danazol— Systemic lupus erythematosus—HIV—Evans' syndrome.

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Autoantibodies in Pediatric Systemic Lupus Erythematosus: Ethnic Grouping, Cluster Analysis, and Clinical Correlations

The Journal of Rheumatology ◽

10.3899/jrheum.080588 ◽

2009 ◽

Vol 36 (2) ◽

pp. 416-421 ◽

Cited By ~ 47

Author(s):

ROMAN JURENCÁK ◽

MARVIN FRITZLER ◽

PASCAL TYRRELL ◽

LINDA HIRAKI ◽

SUSANNE BENSELER ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Cluster Analysis ◽

Ethnic Groups ◽

Ethnic Differences ◽

Lupus Erythematosus ◽

Systemic Lupus ◽

Pediatric Systemic Lupus Erythematosus ◽

Mild Disease ◽

Cluster 2 ◽

Autoantibody Profiles

Objective.(1) To evaluate the spectrum of serum autoantibodies in pediatric-onset systemic lupus erythematosus (pSLE) with a focus on ethnic differences; (2) using cluster analysis, to identify patients with similar autoantibody patterns and to determine their clinical associations.Methods.A single-center cohort study of all patients with newly diagnosed pSLE seen over an 8-year period was performed. Ethnicity, clinical, and serological data were prospectively collected from 156/169 patients (92%). The frequencies of 10 selected autoantibodies among ethnic groups were compared. Cluster analysis identified groups of patients with similar autoantibody profiles. Associations of these groups with clinical and laboratory features of pSLE were examined.Results.Among our 5 ethnic groups, there were differences only in the prevalence of anti-U1RNP and anti-Sm antibodies, which occurred more frequently in non-Caucasian patients (p < 0.0001, p < 0.01, respectively). Cluster analysis revealed 3 autoantibody clusters. Cluster 1 consisted of anti-dsDNA antibodies. Cluster 2 consisted of anti-dsDNA, antichromatin, antiribosomal P, anti-U1RNP, anti-Sm, anti-Ro and anti-La autoantibody. Cluster 3 consisted of anti-dsDNA, anti-RNP, and anti-Sm autoantibody. The highest proportion of Caucasians was in cluster 1 (p < 0.05), which was characterized by a mild disease with infrequent major organ involvement compared to cluster 2, which had the highest frequency of nephritis, renal failure, serositis, and hemolytic anemia, or cluster 3, which was characterized by frequent neuropsychiatric disease and nephritis.Conclusion.We observed ethnic differences in autoantibody profiles in pSLE. Autoantibodies tended to cluster together and these clusters were associated with different clinical courses.

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