Characterization of patients with systemic lupus erythematosus who meet the diagnostic criteria for TAFRO syndrome

Abstract

Purpose TAFRO syndrome is a novel disorder manifesting as fever, anasarca, thrombocytopenia, renal insufficiency and organomegaly, and its etiology has not been clarified. The aim of this study was to elucidate similarities and differences between systemic lupus erythematosus (SLE) and TAFRO syndrome. Methods We examined 46 consecutive patients diagnosed with SLE and determined whether they meet the proposed diagnostic criteria for TAFRO syndrome (2015 version). Results Of the 46 patients with SLE, four (8.7%) also met the TAFRO syndrome criteria (TAFRO-like group). All patients in the TAFRO-like group were males, and their mean age was significantly higher than that of the non-TAFRO group (67.5 ± 8.7 vs. 39.3 ± 18.1 years, p = 0.004). C-reactive protein and γ-glutamyl transpeptidase levels were significantly higher, and frequencies of anti-dsDNA and anti-Sm antibodies were significantly lower in the TAFRO-like than non-TAFRO group. Elder cases (onset age ≥ 50 years) met significantly more categories of the diagnostic criteria for TAFRO syndrome than did those with younger cases. Conclusions Several patients with SLE, especially elder cases, showed features similar to those of TAFRO syndrome. Although exclusion of SLE is needed in the diagnostic criteria for TAFRO syndrome, TAFRO syndrome-like SLE should be considered.

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease

“…These findings highlight that existing iMCD-TAFRO case reports might include patients with undiagnosed autoimmune disorders as suggested previously. [32][33][34][35][36] Thus, clinicians need to carefully exclude those disorders, and the diagnostic criteria need to be defined to avoid misdiagnosis of autoimmune disorders as iMCD-TAFRO.…”

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

“…It should be noted that systemic lupus erythematosus (SLE) sometimes manifests the same characteristics as TAFRO syndrome. Hasegawa et al examined patients with SLE who met the criteria for TAFRO syndrome and found that older patients with SLE (onset ≥ 50 years of age) had clinical features similar to those of TAFRO syndrome [32]. Although anti-SS-A antibody was positive and IgG4 was elevated in the present case, the patient did not meet the criteria for either SjS or IgG4-related disease.…”

TAFRO syndrome: A disease that known is half cured

“…TAFRO syndrome has similar clinical and histopathological characteristics to those of many autoimmune diseases. Approximately 50% of patients often have autoimmune dysfunction 37–39 . For example, rheumatoid‐associated antibodies, platelet‐associated immunoglobulin antibodies, anti‐thyroid antibodies, anti‐Sjögren's syndrome A (anti‐SSA) antibodies, and anti‐SSB antibodies are common immune‐related antibodies in TAFRO syndrome.…”