[Is the current treatment of primary and secondary XXXX effective?].

@entity1 @entity8058 @entity2783 @entity409 @entity305 @entity4932 @entity1582 @entity177 @entity1691 @entity1358 @entity19272 @entity1011 @entity30 @entity309
BACKGROUND: Retrospective study about results of treatment of @entity1 (pts) with primary ( @entity1582 ) and secondary (AA) @entity2783 is given. 31 pts with systemic forms of @entity2783 have been treated and followed-up in our department since 1993. METHODS AND RESULTS: 6 @entity1 and 11 @entity1 were in the @entity1582 group with the mean age of 59 years. @entity1582 was diagnosed in 9 pts, @entity8058 ( @entity8058 ) was found in 8 pts. The kidneys were affected in all pts, heart in 59% of pts, @entity409 and skin in 26% of pts and @entity305 was detected only in 1 pt. Progression of @entity177 with decrease of glomerular filtration rate (GFR) was detected in the @entity1582 group at the end of follow-up period compared with the initial level (p < 0.05) despite the intensive treatment. The difference did not reach statistical significance in other investigated parameters. Median of survival was 13 months from the assessment of diagnosis. Partial remission of @entity2783 was achieved in 9 pts, stable disease was in 5 pts and in 3 pts the disease progressed. 4 @entity1 and 10 @entity1 were in the AA group with mean age of 58 years. Underlying disease was @entity309 in 7 pts, @entity1691 in 2 pts, @entity4932 in 1 pt, @entity30 in 2 pts, @entity19272 in 1 pt and chronic @entity1358 in 1 pt. The kidneys were affected in all pts, bowels and heart in 36% of pts. GFR (p < 0.05) and plasma @entity1011 (p < 0.01) significantly decreased at the end of follow-up period compared with initial levels. Median of survival was 30 months. Partial remission was achieved in 2 pts, stable disease was in 3 pts and progression was detected in 9 pts despite the use of various treatment regimens. CONCLUSIONS: Both forms of @entity1582 represent severe disease with limited response to treatment. The use of new drugs is promising and could lead to better response to treatment.
