[Idiopathic thrombocytopenic purpura as first manifestation of systemic lupus erythematosus lupus].

OBJECTIVES

We studied SLE whose initial symptoms were related to idiopathic thrombocytopenic purpura (ITP) in order to determine if they share clinical and immunologic manifestations.

METHODS

We reviewed the clinical backgrounds of 150 SLE (according to the ACR criteria) periodically followed from 1980 until 1998. We found 12 patients with both these conditions.

RESULTS

All patients were female with a mean age of 32 at the time of ITP diagnosis and 36 at the time of SLE diagnosis. The most usual clinical manifestations were: arthritis (92%), cutaneous (58%) and hematologic involvement with lymphopenia (58%) and thrombocytopenia again (33%) after the initial ITP episode, always together with autoimmune hemolytic anemia (Evans syndrome). None of these patients presented with neurologic involvement and only one presented with renal involvement. 50% were positive for anti-DNA antibodies, 50% were Ro(+) and 16% were RNP (+). 66% were positive for antiphospholipid antibodies and 33% for lupus anticoagulant. Thrombocytopenia was controlled just with steroids in only 16% of the patients. Splenectomy controlled thrombocytopenia with complete remission achieved in 80% (4 from 5) of the patients and 20% (1 from 5) were refractory to this therapy after a medium follow-up time of 6.5 years.

CONCLUSIONS

1) SLE whose initial symptoms are related to ITP were characterized by joints, cutaneous and hematologic involvement without renal and neurologic manifestations. 2) Splenectomy was able to control refractory thrombocytopenia in the majority of these patients.