DOI Seite / Zitierlink:
https://doi.org/10.11588/diglit.48120#0070
52
Molecular and Cell Biology of Autoantibodies and Autoimmunity
The Spectrum of Antinuclear Antibodies (ANA) and Clinical Findings
in Children with Anti-U 1-RNP Antibodies
H. Michels1, H. O. Kettner1, H. Truckenbrodt1, and E. Genth2
1 Rheumatic Children's Hospital, Garmisch-Partenkirchen, FRG
2 Rheumatic Research Institute, Rheumatic Hospital, Aachen, FRG
Since 1977 we have had 30 patients (23 9,7 O') with anti-U 1-RNP antibodies.
All had high titer ANA with a speckled pattern. 19 of the 30 patients had only
anti-U 1-RNP antibodies, 11/30 were found with antibodies to further nuclear an-
tigens:
3 patients to dsDNA,
1 patient to dsDNA and Sm
1 patient to dsDNA, Sm and to S cl-70
3 patients to Sm
1 patient to Sm and SS-A
1 patient to Sm, SS-A and SS-B
1 patient to SS-A
Mean age at onset of disease was 8.4 years, 19/30 had Raynaud's phenomenon,
13/30 recurrent parotid gland enlargements. 24/30 showed skin alterations, often
unspecific maculopapulous exanthema, possible skleroderma-like skin alterations
during further course (13/30). Indication for admission was usually arthritis/ar-
thralgia, mostly from beginning (48%) as polyarthritis.
The arthritis can be deforming, rarely erosive (1/30).
Because of the frequent positive IgM-rheumatoid factor (RF) (21/30), accom-
panied in 7/21 by “rheumatic nodules”, the RF-positive juvenile rheumatoid ar-
thritis must be excluded.
Muscular weakness/myalgia were observed in 39%.
Notable laboratory findings are increased IgG levels (26/30 > 200 mg%, maximal
9440 mg%), often together with elevated total protein (14/26, maximal 12.4%)
and also connected with raised plasma viscosity .
Prognosis depends upon the possible organ manifestations (kidney, lung, cen-
tral nervous system, heart), seems to be favourable, however, quoad vitam in most
cases. So far we have had two fatal cases, a 14-year old girl, with “acute heart
failure” after 8 years disease duration and one 24 year old patient (low titer anti-
U 1-RNP and high titer anti-dsDNA) who died at the age of 25 years, 15 years
after onset of disease during renal transplantation rejection (condition after renal
insufficiency through membranoproliferative glomerulonephritis).
It seems that the titer level of anti-U 1-RNP antibodies and the spectrum of
ANA bear significance for the extent of the clinical symptomatology.
Molecular and Cell Biology of Autoantibodies and Autoimmunity
The Spectrum of Antinuclear Antibodies (ANA) and Clinical Findings
in Children with Anti-U 1-RNP Antibodies
H. Michels1, H. O. Kettner1, H. Truckenbrodt1, and E. Genth2
1 Rheumatic Children's Hospital, Garmisch-Partenkirchen, FRG
2 Rheumatic Research Institute, Rheumatic Hospital, Aachen, FRG
Since 1977 we have had 30 patients (23 9,7 O') with anti-U 1-RNP antibodies.
All had high titer ANA with a speckled pattern. 19 of the 30 patients had only
anti-U 1-RNP antibodies, 11/30 were found with antibodies to further nuclear an-
tigens:
3 patients to dsDNA,
1 patient to dsDNA and Sm
1 patient to dsDNA, Sm and to S cl-70
3 patients to Sm
1 patient to Sm and SS-A
1 patient to Sm, SS-A and SS-B
1 patient to SS-A
Mean age at onset of disease was 8.4 years, 19/30 had Raynaud's phenomenon,
13/30 recurrent parotid gland enlargements. 24/30 showed skin alterations, often
unspecific maculopapulous exanthema, possible skleroderma-like skin alterations
during further course (13/30). Indication for admission was usually arthritis/ar-
thralgia, mostly from beginning (48%) as polyarthritis.
The arthritis can be deforming, rarely erosive (1/30).
Because of the frequent positive IgM-rheumatoid factor (RF) (21/30), accom-
panied in 7/21 by “rheumatic nodules”, the RF-positive juvenile rheumatoid ar-
thritis must be excluded.
Muscular weakness/myalgia were observed in 39%.
Notable laboratory findings are increased IgG levels (26/30 > 200 mg%, maximal
9440 mg%), often together with elevated total protein (14/26, maximal 12.4%)
and also connected with raised plasma viscosity .
Prognosis depends upon the possible organ manifestations (kidney, lung, cen-
tral nervous system, heart), seems to be favourable, however, quoad vitam in most
cases. So far we have had two fatal cases, a 14-year old girl, with “acute heart
failure” after 8 years disease duration and one 24 year old patient (low titer anti-
U 1-RNP and high titer anti-dsDNA) who died at the age of 25 years, 15 years
after onset of disease during renal transplantation rejection (condition after renal
insufficiency through membranoproliferative glomerulonephritis).
It seems that the titer level of anti-U 1-RNP antibodies and the spectrum of
ANA bear significance for the extent of the clinical symptomatology.