Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis

Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile...

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Published inCurēus (Palo Alto, CA) Vol. 11; no. 6; p. e4929
Main Authors Kasparian, Saro, Anand, Kartik, Burns, Ethan, Chung, Betty, Pingali, Sai Ravi Kiran
Format Journal Article
LanguageEnglish
Published United States Cureus Inc 18.06.2019
Cureus
Subjects
Arthritis
Autoimmune diseases
Bone marrow
Case reports
Cytotoxicity
Etiology
Family medical history
Hepatitis
Hospitals
Infections
Liver
Lymphocytes
Mortality
Oncology
Other
Patients
Pediatrics
Population
Rheumatology
Sarcoidosis
Steroids
Transplants & implants
United States > US
macrophage activation syndrome
treatment
sarcoid
hlh
extrapulmonary sarcoidosis
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Summary:Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile idiopathic arthritis. MAS occurring as a sequela of sarcoidosis is seldom reported. Herein, we present an uncommon case of MAS occurring secondary to suspected extrapulmonary sarcoidosis and the associated diagnostic challenges. A 53-year-old White female presented with a 20-month history of constitutional symptoms of an unclear etiology. Her extensive workup included equivocal bone marrow and liver biopsies, suggestive of occasional hemophagocytosis. On admission, she met criteria for HLH based on the HLH-94 diagnostic guidelines. A repeat liver biopsy was performed revealing non-necrotizing granulomas in the parenchyma. Given the concern for an extrapulmonary sarcoidosis, she was started on pulse-dose steroids with subsequent symptomatic resolution. Two years later, she remains in complete remission. As a systemic disease, sarcoidosis can manifest in any organ and present in a variety of ways. While HLH and MAS have numerous etiologies, sarcoidosis should be considered as a potential underlying diagnosis, and prompt treatment initiation with steroids may reduce morbidity and mortality.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.4929