Objective: Determination of the prevalence of severe hematologic manifestations among Filipino patients with systemic lupus erythematosus (SLE) and analysis of any association with organ involvement and serology.

Methods: This cross-sectional study included SLE patients 19 years old and above seen at the UST Hospital from 2012 to 2017. Patients with severe hematologic manifestations (severe hemolytic anemia, severe thrombocytopenia, and Evans syndrome (ES)) were identified and their prevalence determined. Independent t-test was used to compare continuous variables. Categorical variables were measured using the chi-square test; odds ratios (OR) with their corresponding 95% confidence interval were calculated using the SPSS software version 21. This study has been approved by the Institutional Review Board.

Results: Of the 253 patients (238 females, 94.07%), the mean age at diagnosis was 27.04 (SD 9.96) years. Severe hematologic involvement was noted in 12.26% (n=31); severe hemolytic anemia was the most prevalent (14, 5.53%), followed by severe thrombocytopenia (13, 5.14%) and ES (4, 1.58%). Higher prevalence of major organ involvement was observed among patients who manifest with severe hematologic disease. Severe thrombocytopenia was more likely to have cardiac involvement (OR 7.39, 95% CI 1.90 to 28.81, p=0.004). A higher prevalence of negative baseline anti-dsDNA serology was seen among patients who developed ES.

Conclusion: Severe hematologic involvement was noted in 12.26% of Filipino SLE patients, with hemolytic anemia as the most frequently recorded. Severe thrombocytopenia was associated with cardiac involvement among these patients. Patients developing ES tend to have a negative anti-dsDNA serology.

Lupus Erythematosus, Systemic ; Anemia, Hemolytic ; Thrombocytopenia

INTRODUCTION

Macrophage Activation Syndrome (MAS) is a rare but life threatening pro-inflammatory complication of multiple autoimmune diseases leading to cytokine storm. We report a case of MAS as a presenting manifestation of Systemic Lupus Erythematosus.

CASE REPORT

A 32-year-old female, newly diagnosed with Systemic Lupus Erythematosus (SLE), presents with a 3-month history of fever and joint pains, which began a few days after receiving her first dose of a viral vector COVID-19 vaccine. She later developed facial edema, and her fever became persistent and unremitting. Upon presentation, she was initially hypotensive, tachycardic, with distended neck veins, with periorbital edema and muffled heart sounds. Initial work-up revealed pericardial effusion, anemia, thrombocytopenia, elevated creatinine, hypoalbuminemia, hematuria, and pyuria. She was intubated, started on inotrope, and underwent pericardiocentesis. Patient was classified as SLE based on Systemic Lupus International Collaborating Clinics Classification (SLICC) Criteria despite negative antinuclear antibody (ANA). Nevertheless, she was started on IV steroids and hydroxychloroquine. She was eventually extubated after significant clinical improvement. Further work-up for MAS was however done due to persistent febrile episodes. Hyperferritinemia, hypertriglyceridemia, hypercholesterolemia, pancytopenia, transaminitis, and splenomegaly on imaging were noted. She was then started on methylprednisolone pulse therapy. After treatment, marked clinical improvement, as well as resolution of transaminitis and pancytopenia were noted.

CONCLUSION

A high index of suspicion for MAS should exist in a patient with pyrexia of unknown origin with concomitant autoimmune disease. In this disease that can lead to progressive organ failure, early diagnosis and management is crucial. This case report culminates the need for diagnostic and therapeutic guidelines that will help in the early diagnosis and immediate treatment of this debilitating condition.

Human ; Female ; Adult: 25-44 Yrs Old ; Lymphohistiocytosis, Hemophagocytic ; Macrophage Activation Syndrome ; Lupus Erythematosus, Systemic ; Autoimmune Diseases