To define the clinical manifestations,
autoantibody associations, optimal treatment, and prognosis of hypergammaglobulinemic
purpura associated with systemic autoimmune
rheumatic diseases.
METHODS: Of 303 consecutive patients with systemic autoimmune
rheumatic diseases evaluated over 5 years, 17 French Canadian patients with hypergammaglobulinemic
purpura with
systemic lupus erythematosus (SLE) (n = 12) or another systemic autoimmune
rheumatic disease (n = 5) were identified and followed prospectively. Mild secondary Sjörgren's syndrome developed in 9 (53%) patients.
RESULTS: Sixteen (94.1%) patients were women. Attacks of hypergammaglobulinemic
purpura occurred in the pretibial (76.5%) or perimalleolar (70.5%) areas or the dorsal aspect of the feet (52.9%). Triggering factors included walking, prolonged standing, and alcohol intake. The mean duration of attacks was 6.1 days. Systemic manifestations consistent with a flare of the underlying systemic autoimmune
rheumatic diseases accompanied hypergammaglobulinemic
purpura attacks in 15 (88%) patients.
Arthralgias (n = 13, 86.6%),
arthritis (n = 9, 69.2%), and
periarthritis were characterstically localized adjacent to the
purpura. Anti-
Ro antibodies were expressed in all (100%) patients with hypergammaglobulinemic
purpura with SLE, but in only 11 (28.9%) of 38 consecutive patients with SLE without hypergammaglobulinemic
purpura (P < 0.000001, odds ratio 84, 95% confidence interval 4.6, 1525). The positive predictive values for hypergammaglobulinemic
purpura in SLE were: anti-Ro plus anti-La 73%, anti-La 57%, and anti-Ro 52%. The negative predictive value of anti-Ro was 100%. Although 11 (92%) patients with SLE with anti-Ro expressed anti-52 kDa Ro [4 (36.3%) of whom also expressed anti-60 kDa Ro], this frequency was not greater than in anti-Ro positive patients with SLE without hypergammaglobulinemic
purpura. The effects of
indomethacin or
hydroxychloroquine were assessed over 6 months in 8 patients with recurrent incapacitating hypergammaglobulinemic
purpura. Complete (n = 4) or partial (n = 4) remission of hypergammaglobulinemic
purpura occurred. In 5 additional patients with severe hypergammaglobulinemic
purpura, attacks stopped with
prednisone 25 to 60 mg daily. The mean duration of hypergammaglobulinemic
purpura followup was 5.4 years (range 1-6 years). At last followup, hypergammaglobulinemic
purpura had resolved in 11 (64.7%) patients despite persistently abnormal serology.
CONCLUSION: