HYPERGAMMAGLOBULINEMIC PURPURA IN SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES - PREDICTIVE VALUE OF ANTI-RO(SSA) AND ANTI-LA(SSB) ANTIBODIES ANDTREATMENT WITH INDOMETHACIN AND HYDROXYCHLOROQUINE

Objective, To define the clinical manifestations, autoantibody associa tions, optimal treatment, and prognosis of hypergammaglobulinemic purp ura associated with systemic autoimmune rheumatic diseases. Methods, O f 303 consecutive patients with systemic autoimmune rheumatic diseases evaluated over 5 years, 17 French Canadian patients with hypergammagl obulinemic purpura with systemic lupus erythematosus (SLE) (n = 12) or another systemic autoimmmune rheumatic disease (n = 5) were identifie d and followed prospectively. Mild secondary Sjogren's syndrome develo ped in 9 (53 %) patients. Results, Sixteen (94.1 %) patients were wome n. 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, Syste mic manifestations consistent with a flare of the underlying systemic autoimmune rheumatic diseases accompanied hypergammaglobulinemic purpu ra attacks in 15 (88 %) patients. Arthralgias (n = 13, 86.6 %), arthri tis (n = 9, 69.2 %), and periarthritis were characteristically localiz ed adjacent to the purpura. Anti-Re antibodies were expressed in all ( 100 %) patients with hypergammaglobulinemic purpura with SLE, but in o nly 11 (28.9 %) of 38 consecutive patients with SLE but without hyperg ammaglobulinemic purpura (p < 0.000001, odds ratio 84, 95 % confidence interval 4.6, 1525). The positive predictive values for hypergammaglo bulinemic purpura in SLE were: anti-Re plus anti-La 73 %, anti-La 57 % , and anti-Ro 52 %. The negative predictive value of anti-Re was 100 % . Although 11 (92 %) patients with SLE with anti-Iio expressed anti-52 kDa Ro [4 (36.3 %) of whom also expressed anti-60 kDa Re], this frequ ency was not greater than in anti-Re positive patients with SLE withou t hypergammaglobulinemic purpura, The effects of indomethacin or hydro xychloroquine were assessed over 6 months in 8 patients with recurrent incapacitating hypergammaglobulinemic purpura. Complete (n = 4) or pa rtial (n = 4) remission of hypergammaglobulinemic purpura occurred. In 5 additional patients with severe hypergammaglobulinemic purpura, att acks stopped with prednisone 25 to 60 mg daily, The mean duration of h ypergammaglobulinemic 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, In the absence of anti-Iio antibodies, a presumptive diagnosis of hype rgammaglobulinemic purpura secondary to SLE should be questioned. Pred nisone should be used only in severe hypergammaglobulinemic purpura. I ndomethacin and hydroxychloroquine are of value in the treatment of mi lder hypergammaglobulinemic purpura.