Case Presentation: 17 year old female with no medical history presented with malaise. Labs were WBC 3.78 with normal differential, a microcytic anemia 7.1, platelets 7, and uric acid 10.6. On admission, she was found to have epistaxis, conjunctival hemorrhage, and hemoptysis. She required escalation to high flow nasal cannula. Chest x-ray was concerning for interstitial infiltrates to bilateral middle lobes concerning for diffuse alveolar hemorrhage. Due to severe anemia and thrombocytopenia, massive transfusion protocol was activated. Peripheral smear demonstrated 3 schistocytes. Hematology consulted, who were concerned for TTP due to high PLASMIC score of 7. Hematology recommended methylprednisolone and IVIG. Patient decompensated and required intubation for acute hypoxic respiratory failure. In light of clinical deterioration, persistent thrombocytopenia, mucosal bleeding, and new acute renal injury, PLEX therapy was initiated. ADAMTS13 was negative. ANA was positive with titer of 1:640, C3 of 47, C4 of 6, ANCA +, and p-ANCA 1:320. Rheumatology was consulted who recommended pulse steroids and PLEX. She required CCRT and nicardipine drip for blood pressure control as systolic pressures were consistently in 150s range. With hypertension and slow recovery of renal function, concern for lupus nephritis arose therefore renal biopsy was pursued once downgraded to floor. Biopsy revealed class lll lupus nephritis with active lesions, endocapillary hypercellularity, and neutrophils/karyohexis in 41% of glomeruli sampled. While on the pediatric floor, course was complicated by mental status changes and protracted return to baseline neurological status. Started hydroxychloroquine and transitioned to oral steroids upon discharge.
Discussion: Juvenile-onset systemic lupus erythematous is an autoimmune disease characterized by multiorgan involvement prior to 18 years old. Pediatric patients demonstrate a more variable presentation and clinical course when compared to adults with a higher prevalence of lupus nephritis and hematologic abnormalities. Other differences compared to adult onset disease include higher disease activity, earlier organ damage, and increased use of immunosuppressive treatment. Mild thrombocytopenia is an initial finding in 15% of pediatric cases. Severe thrombocytopenia is rare and has been more so correlated with hemorrhagic complications, such as diffuse alveolar hemorrhage. This is an atypical case of an adolescent mistakenly diagnosed with thrombocytopenia purpura (TTP), which delayed attaining the correct diagnosis of jSLE. The patient did not present with physical exam findings that were suggestive of lupus. Patient required aggressive immunosuppressive therapy including IV steroids, administration of IVIG, and eight total PLEX sessions throughout admission. Patient’s course was further complicated by CCRT due to acute renal failure, AMS, and intubation. Once the differential was broadened to include rheumatological processes, the patient satisfied 4/17 SLICC criteria, and a kidney biopsy confirmed Class lll lupus nephritis.
Conclusions: This case iterates the importance of early recognition of thrombocytopenia being the presenting symptom in pediatric SLE patients in the absence of other classical physical manifestations of lupus as expected in adult cases. Earlier diagnosis in our patient’s case would have led to sooner initiation of immunosuppressant agents, which likely would have circumvented progression of end organ damage.
