Background/Purpose: Systemic lupus erythematosus (SLE), a chronic inflammatory autoimmune disease capable of exhibiting virtually any clinical symptoms, occasionally presents itself with thrombocytopenic purpura as an initial manifestation. Some patients with initial diagnosis of idiopathic thrombocytopenic purpura (ITP) develop symptoms of SLE as time goes by. Our aim was to investigate the prevalence of SLE in patients with the initial diagnosis of ITP and recognize the salient characteristics of these patients, thereby identifying early diagnostic clues.

Methods: We retrospectively analyzed the clinical and laboratory features of 337 (203 females, 134 males) patients initially diagnosed with ITP between January 1993 and April 2012 at a single center, Kyung Hee University Hospital. At the time of diagnosis of ITP, patients with autoimmune diseases were excluded and ITP was classified into chronic, acute, and recurrent acute types. The following data were obtained for every patient: age at diagnosis, sex, presence of ANA and anti-platelet antibody, bone marrow findings, and bleeding manifestations. The patients were followed-up for development of symptoms indicative of SLE and clinical events including immune hemolytic anemia, splenectomy, infections and thrombosis.

Results: Fifteen of the 337 patients (4.5%) developed SLE; 14 were females while all 15 showed ANA positivity. The patients who eventually developed SLE were more likely to have chronic type ITP, higher ESR, and speckled type ANA, irrespective of its titer (p< 0.05, respectively). The mean incubation period turned out to have SLE was 33.3 months (range: 2-159). For these patients, the mean age at initial diagnosis of ITP was 26.6 years (range: 13-65), and the mean age at diagnosis of SLE was 29.4 years (range: 15-66). Splenectomy was performed in 4 patients, and 3 of them suffered from at least one event of infection. Six of the 15 ITP patients with later development of SLE had been diagnosed as having Evan’s syndrome (ITP with immune hemolytic anemia). The most notable SLE manifestations were thrombotic complications such as deep vein thrombosis or cerebral infarction (40%), and accordingly, high positivity of anti-phospholipid antibody (6/13, 46.2%). Contrary to previous reports, renal involvement appeared to be quite frequent (7/15, 46.6%).

Conclusion: SLE was developed in 4.5% of patients with initial diagnosis of ITP during follow-up. ITP patients with later development of SLE are associated with adverse clinical course, therefore early recognition and accurate interventions are imperative. The presence of speckled type ANA, elevated ESR or immune hemolytic anemia in ITP patients warrant careful monitoring, as they are prone to develop other manifestations of SLE or thrombotic complications. With increased clinical attention, unnecessary invasive procedures, such as splenectomy, can be avoided and timely initiation of disease-specific treatment possible.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-for-future-development-of-systemic-lupus-erythematosus-in-korean-patients-with-idiopathic-thrombocytopenic-purpura/