SHM Converge Scientific Abstract ePoster Gallery-Delayed Diagnosis of Juvenile Sle With Severe Thrombocytopenia and Diffuse Alveolar Hemorrhage

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This case describes a 17-year-old previously healthy female with malaise who presented with severe thrombocytopenia, microcytic anemia, leukopenia, epistaxis, conjunctival hemorrhage, and hemoptysis. Initial labs showed platelets of 7, hemoglobin 7.1, and elevated uric acid. Chest imaging revealed bilateral interstitial infiltrates concerning for diffuse alveolar hemorrhage (DAH), and she required intubation. Because of her severe cytopenias, mucosal bleeding, acute kidney injury, and a high PLASMIC score, she was initially treated for thrombotic thrombocytopenic purpura (TTP) with plasma exchange.<br /><br />Further evaluation showed ADAMTS13 was negative, while autoimmune testing was strongly suggestive of systemic lupus erythematosus (SLE), including positive ANA (1:640), low complement levels, and positive p-ANCA. Rheumatology recommended pulse steroids and continued plasmapheresis. Her course was complicated by renal failure requiring CRRT and hypertension requiring nicardipine. Renal biopsy later demonstrated class III lupus nephritis with active lesions.<br /><br />After delayed recognition of juvenile-onset SLE (jSLE), she was started on hydroxychloroquine and transitioned to oral steroids at discharge. Her recovery was prolonged by delayed neurologic return to baseline.<br /><br />The report highlights that jSLE often presents differently from adult-onset disease, with more hematologic abnormalities and lupus nephritis, but sometimes without classic physical findings. Severe thrombocytopenia can be an early manifestation and may be associated with life-threatening hemorrhagic complications such as DAH. This case emphasizes the importance of considering autoimmune causes early when adolescents present with isolated thrombocytopenia and bleeding, to avoid delay in immunosuppressive treatment and prevent organ damage.

Asset Subtitle

Alexia R. Jauregui

Meta Tag

Author List Alexia R. Jauregui, Upulie Nawaratne

Category Clinical Vignettes

Concept Juvenile-onset systemic lupus erythematosus

Concept Thrombocytopenia

Concept Thrombotic Thrombocytopenic Purpura

Concept Diffuse Alveolar Hemorrhage

Concept Renal involvement

Distinguished Non-Finalist

Presenter Organization The University of Texas at Houston Health Sciences Center

Presenting Author Alexia R. Jauregui

Track Pediatric

Keywords

juvenile-onset systemic lupus erythematosus

severe thrombocytopenia

diffuse alveolar hemorrhage

thrombotic thrombocytopenic purpura

plasma exchange

lupus nephritis

ADAMTS13 negative

autoimmune cytopenias

hemoptysis

hydroxychloroquine

Juvenile-onset systemic lupus erythematosus

Thrombocytopenia

Thrombotic Thrombocytopenic Purpura

Diffuse Alveolar Hemorrhage

Renal involvement