Case Presentation: A 19 year old female with a history of systemic lupus was found at home unresponsive by family and brought to a local ED. At the ED she was hyperthermic to 106F and hypotensive then transferred to Vidant Medical Center fevers and septic shock. Of note she had recently been discharged from Duke University Hospital for a presumed lupus flare and was not transferred there due to lack of ICU beds at the time. CT chest and abdomen had new bulky thoracic, abdominal, and axillary lymphadenopathy. There was concern for a lupus flare as her prior admission had a similar presentation and steroids were started. After two days she became hypoxic and agitated and was intubated. She was treated for respiratory failure and septic shock deemed secondary to an E. Coli UTI and MSSA pneumonia. Oncology was consulted for the lymphadenopathy and patient had an axillary lymph node biopsy performed after which she was moved to a general bed. On the medicine service she suddenly developed seizures and CT head confirmed newly found PRES. Patient was moved back to the medical ICU awaiting transfer to Duke. Labs had elevated ESR (31), CRP (77), and INR=1.6. Ferritin was significantly elevated at >40,000. Hypocomplementemia was noted. Her Echocardiogram prior to being transferred show global hypokinesis of the LV and EF of 30%. Rheumatology was consulted and her multiorgan failure and presentation was atypical for Lupus and was concerned she had an evolving variant of Macrophage Activating Syndrome (MAS). She was transferred to Duke and ultimately her excisional biopsies revealed an underlying diagnosis of Multicentric Castleman Disease (MCD) which was likely the underlying etiology of her MAS. Her disease eventually responded to cyclosporine and an siltuximab.
Discussion: MCD has been described as the great mimic given its ability to present like many other rheumatalogic and oncologic cases. While multiple case reports exist for MCD mimicking lupus, few present with concurrent MAS. Diagnosing prior to pathology requires a high index of suspicion due to nonspecific symptoms and low prevalence, especially in HHS-8 negative and HIV negative patients. Lab criteria involve the H score which calculates the probability of having the disease. This patient’s H score was >250 giving a probability of >99%. While this patient had a high score, the final pathology confirmed diagnosis. At our institution, patient was seen by rheumatology, neurology, nephrology, hematology oncology, and surgical oncology but diagnosis was not made until the pathologist was able to evaluate tissue. This case is also important because it shows the importance of taking ownership when a patient is managed by another institution. When the patient arrived at our hospital, the very first note mentioned transferring to Duke because patient’s care was already managed there including a recent admission for a presumed lupus flare. However, the decision was made to take ownership instead of waiting for a transfer and these steps gave the diagnosis. Patient eventually improved and left AMA once her mental status improved.
Conclusions: This case of a patient who presented with multisystem organ failure secondary to macrophage activating syndrome from an undiagnosed Castleman disease. Her case demonstrates the importance of keeping a broad differential even when a patient has been carrying another diagnoses prior to admission from another tertiary care center. Castleman Disease may mimic lupus however making the correct diagnosis is critical to the patient’s clinical course.