Abstract Number: PB1923
Meeting: ISTH 2020 Congress
Theme: Thrombotic Microangiopathies » Antiphospholipid Syndrome
Background: In 2005, we published the case of a 30-year-old man with a rare association of Kikuchi-Fujimoto Disease (KFD) and a definitive Catastrophic Antiphospholipid Syndrome (CAPS), triple-positive at high titers for antiphospholipid antibodies (aPL). Cytomegalovirus infection was the triggering factor. The patient presented severe distal necrosis in the four extremities. Among other therapies, he was treated with recombinant activated protein C, which probably improved his altered coagulation profile. He evolved satisfactorily and has been under oral vitamin K antagonist anticoagulants (RIN=3-3.5) and glucocorticoids since then.
Aims: During the next 10 years, he suffered at least 3 relapses/year, which always included high erythrocyte sedimentation rate, liver-splenomegaly, aPL persistence and 1-2 of the characteristics shown in table 1. Strikingly, other autoantibodies were negative. All these episodes were treated successfully with a higher dose of oral glucocorticoids (20 mg/day).
Methods: According to previous reports, some KFD evolved to an autoimmune disease, particularly systemic lupus erythematous. However, in this case it was not until 2015 that the diagnosis could be achieved: the patient accumulatively presented enough characteristics to define adult-onset Still disease (AOSD) (Yamaguchi criteria). In addition, ferritin levels were increased (1800 mg∕dl). Infections, malignancies and connective tissue diseases were excluded.
Results: The patient was treated with methotrexate, azathioprine, mycophenolate and/or leflunomide, but without success. Indeed, the frequency of the crisis increased to 5-6 episodes/year and were responsive to higher oral dose of corticosteroids.
Conclusions: Despite the high mortality rate of CAPS and the usual prognosis of KFD into an autoimmune pathology, this patient successfully survived and just 10 years later his pathology was properly identified as a systemic inflammatory syndrome known as AOSD. The only autoantibodies detected were persistently triple positivity of aPL, always to high titers, without thrombosis. As with current therapies the patient has a poor prognosis we are evaluating to apply a biological treatment.
| Clinical | Laboratory |
| – High temperature of more than two weeks of evolution (peaks) | – Leukocytosis with neutrophilia |
| – Maculopapular rash | – Increased liver enzymes |
| – 2nd and 3rd right metacarpo-phalangical synovitis; left elbow synovitis | |
| – Odynophagia | |
| – Diarrhea and vomiting | |
| – Poly-arthralgias |
[Table 1: Clinical and laboratory characteristics of the crisis alternatively presented between 2005 – 2015.]
| Major criteria |
| 1. Fever of 39ᵒC or higher (≥1 week) |
| 2. Arthralgia (≥2 weeks) |
| 3. Salmon colored maculopapular rash |
| 4. Leukocytosis (≥10.000∕ul with ≥80% granulocytes) |
| Minor criteria: |
| 1. Sore throat |
| 2. Lymphadenopathy and∕or splenomegaly |
| 3. Liver dysfunction |
| 4. Negative rheumatoid factor and antinuclear antibody test. |
[Table 2. Yamaguchi criteria (1992): five features, including at least two major criteria, must be present for adult-onset Still disease diagnosis.]
To cite this abstract in AMA style:
Peres SDA, Remondino G, Aranda F, Lucero A, Lencinas G, Cunto E, de Larrañaga GF. Catastrophic Antiphospholipid Syndrome, Kikuchi-Fujimoto Disease and Adult-Onset Still Disease: An Unusual Association [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/catastrophic-antiphospholipid-syndrome-kikuchi-fujimoto-disease-and-adult-onset-still-disease-an-unusual-association/. Accessed September 22, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/catastrophic-antiphospholipid-syndrome-kikuchi-fujimoto-disease-and-adult-onset-still-disease-an-unusual-association/