Catastrophic antiphospholipid syndrome and Kikuchi-Fujimoto disease: the first case reported

Abstract

The case of a man with diagnosis of Kikuchi-Fujimoto disease (KFD) and catastrophic antiphospholipid syndrome (CAPS) is reported. He presented prolonged fever, lymphadenopathies, arthralgia, weight loss, hepatosplenomegaly and positive IgM for cytomegalovirus. While he was empirically treated with tuberculostatic drugs, he suddenly developed systemic inflammatory response syndrome, multiple organ failure and distal necrosis. On suspicion of severe sepsis, antibiotics, corticoids and recombinant human activated protein C (XIGRIS) were administrated. Exhaustive laboratory searching was negative. Histopathologic examinations of lymph node first disclosed malignant lymphoma but later KFD was confirmed. One month later, laboratory tests showed the presence of antiphospholipid antibodies (aPL). The patient was discharged after two months of hospitalization. This case exhibits a KFD complicated by definite CAPS. Cytomegalovirus could be involved in the development of both CAPS and KFD. Because of the severity of the case, we believe that XIGRIS noticeable improved the altered coagulation profile associated with CAPS.

Kikuchi-Fujimoto Disease in a 25-Year-Old Female: A Case Report

“…Other symptoms include fevers, arthralgia, night sweats, arthritis, and rash [ 8 ]. Additional case reports reported other clinical presentations such as pleural effusion [ 9 ], hemolytic uremic syndrome (HUS) [ 10 ], and antiphospholipid syndrome with multiorgan failure [ 11 ]. Laboratory abnormalities vary between cases.…”

Kikuchi-Fujimoto Disease Complicated by Rheumatoid Arthritis, Type 1 Diabetes Mellitus, and Hypothyroidism

“…It was initially described in 1972 simultaneously by Masahiro Kikuchi and Fujimoto et al in two Japanese women with cervical lymphadenopathy, low-grade fever, rash, and arthralgias [2][3]. KFD also commonly causes diffuse rash, arthritis, fatigue, and hepatosplenomegaly, while it less commonly causes thyroiditis, encephalitis, seizures, pleuritis, pericarditis, hepatitis, and anti-phospholipid syndrome leading to multi-organ failure [7,8,[9][10][11][12]. Laboratory and serologic studies are often normal but may show leukopenia (in 43% of patients), thrombocytopenia, anemia, or atypical lymphocytosis (in 25% of patients).…”

Cytomegalovirus‐induced cutaneous microangiopathy manifesting as lower limb ischemia in a human immunodeficiency virus‐infected patient

“…Interestingly, the mechanism of antiphospholipid antibody production in the setting of CMV infection has been carefully elucidated and involves partial reactivation of CMV with enhancement of apoptosis, a critical event in the induction of antibodies to cytoplasmic membrane‐based phospholipids. Indeed, there are many reports addressing catastrophic thrombotic states in this setting . Vasculopathic change associated with CMV infection in the absence of inclusion bodies has also been described and is postulated to define the basis of rapidly progressive coronary artery disease and endothelialitis in cardiac transplant patients, coronary restenosis after angioplasty and inflammatory aortic disease .…”

“…Indeed, there are many reports addressing catastrophic thrombotic states in this setting. 19 Vasculopathic change associated with CMV infection in the absence of inclusion bodies has also been described and is postulated to define the basis of rapidly progressive coronary artery disease 20 and endothelialitis in cardiac transplant patients, 21 coronary restenosis after angioplasty 22 and inflammatory aortic disease. 23,24 The vasculopathy is held to represent the sequelae of partial reactivation with synthesis of viral proteins that influence the cellular regulation of other genes in the infected cell but do not participate in viral synthesis.…”