Background: The treatment of Kawasaki disease is controversial when intravenous immunoglobulin therapy fails, although it typically relies on combinations of prednisolone, infliximab, cyclosporine, and plasma exchange therapy. The goal of the treatment is no longer merely to reduce mortality but also to decrease the sequelae of coronary artery lesions, which are the most common and potentially life-threatening complications. Recently, plasma exchange therapy has been used to treat intravenous immunoglobulin-unresponsive Kawasaki disease with coronary artery lesions. When performed before coronary artery dilatation, the outcomes for plasma exchange are known to be excellent; however, when dilatation is already present, sequelae persist. Methods: Between December 2006 and April 2015, we treated ten patients with Kawasaki disease complicated by coronary artery lesions that received plasma exchange because intravenous immunoglobulin therapy had proven to be ineffective. Here, we retrospectively review the efficacy and safety of plasma exchange therapy in such unresponsive cases against coronary artery lesions in patients with Kawasaki disease when plasma exchange performed after coronary artery dilatation. Results: In nine of the ten patients (90.0%), the body temperature was confirmed to be < 37.5°C at an average of 2.7 ± 1. 4 days after starting plasma exchange. Serum C-reactive protein levels decreased significantly from 9.9 ± 4.9 mg/dL before exchange to 1.9 ± 2.9 mg/dL after exchange (P < 0.05). One year after plasma exchange treatment, the coronary artery lesions had regressed to within normal limits in six of the ten patients. Although lesions remained in three patients, all three of these patients were asymptomatic. In addition, there were no stenosis of the coronary artery in nine of the ten patients. One patient died due to a ruptured giant coronary aneurysm 1 day after starting plasma exchange. Conclusions: In conclusion, plasma exchange may be effective in not only regressing coronary artery lesions but also preventing sequelae in patients with Kawasaki disease when plasma exchange is performed after coronary artery dilatation.
Leukocyte apheresis (LCAP) is a safe and effective treatment for active ulcerative colitis (UC) in Japan. Nevertheless, a limitation of LCAP is its requirement for two puncture sites (double‐needle [DN] apheresis), sometimes leading to problems with needle puncture. Single‐needle (SN) apheresis is useful in hemodialysis and reduces needle puncture pain. If SN apheresis were found to be useful in LCAP for UC, it may reduce patient burden. The aim of this study was to compare the safety and efficacy of SN apheresis with that of DN apheresis. Twenty‐four patients with active UC were retrospectively enrolled. They underwent either SN apheresis (n = 12) or conventional double‐needle (DN) apheresis (n = 12) at the Kurume University Hospital from February 2014 to March 2018. At each session, we recorded access problems defined by the time required to initiate apheresis and the frequency of puncture‐related problems, as well as blood circuit clotting, defined as clotting necessitating interruption of apheresis and changing of the circuit. Efficacy was assessed using partial Mayo scores. The number of apheresis sessions was comparable between SN and DN apheresis (9.0 ± 2.0 times vs 9.6 ± 1.4 times, mean ± SEM). SN significantly reduced the time required to start apheresis (10.0 ± 5.4 minutes vs 19.4 ± 11.9 minutes, P < .05) as well as needle puncture troubles (0.9% vs 11.5%, P < .05). SN had comparable frequency of blood clotting episodes (5.6% vs 8.7%). SN apheresis had similar clinical efficacy (P < .001 in SN and P < .01 in DN). The improvement and remission rates were comparable between groups. SN apheresis may be safe and effective and may reduce patient burden during UC treatment. Nevertheless, further comparative studies are needed.
A 65-year-old male patient with nephrotic syndrome was admitted to our hospital due to worsening systemic edema and purpura on the limbs. He had an impaired renal function, low serum complement level, and elevated rheumatoid factor level. He was positive for cryoglobulin (monoclonal IgM-κ and polyclonal mixed-type IgG), and the results of his kidney biopsy showed a tissue profile of membranoproliferative glomerulonephritis (MPGN). Due to the fact that the secondary cause was unclear, he was diagnosed with MPGN due to essential mixed cryoglobulinemia. On hospital day 20, he was initiated on 50 mg/day prednisolone (PSL). On hospital day 43, oral mizoribine (MZR) at a dose of 150 mg/day was prescribed. On hospital day 49, cryofiltration was performed because the disease was steroid resistant. The treatment promptly decreased urine protein levels. Serum albumin and serum complement levels increased, and complete remission was achieved approximately three months after the initiation of treatment. The PSL and MZR doses were gradually reduced to 2 mg/day and 100 mg/ day, respectively, without any reemergence of the symptoms of cryoglobulinemia or relapse of the nephrotic syndrome for three years. Here, we report this case with essential mixed cryoglobulinemia in whom we could achieve complete remission of the disease by adding cryofiltration to the oral corticosteroid and immunosuppressant therapy with mizoribine and could maintain for a long time.
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