Resistant oral mucosal lesions in pemphigus vulgaris responsive to double filtration plasmapheresis: First case report from Turkey

Şavk, Ekin; Uslu, Meltem; Yavaşoğlu, İrfan; Kadıköylü, Gürhan; Şendur, Neslihan; Karaman, Göksun

doi:10.1016/j.transci.2013.01.008

Cited by 5 publications

(9 citation statements)

References 13 publications

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“…Since the successful treatment of pemphigus by plasmapheresis was first reported in 1978 , plasmapheresis and double‐filtration plasmapheresis (DFPP), which is more selective and safer, have been widely adopted. Both treatments have been covered by health insurance in Japan since 1992 . Here we aimed to evaluate the efficacy of DFPP for the treatment of five patients with drug‐resistant pemphigus.…”

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confidence: 99%

Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

et al. 2017

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Double-filtration plasmapheresis is an effective and safe treatment for pemphigus. We retrospectively evaluated the decrease in autoantibody titer and pemphigus disease area index following double-filtration plasmapheresis in five patients with moderate to severe pemphigus, who were physically and/or serologically unresponsive to 1.0 mg/kg per day of prednisolone and other supportive drugs and ointments. The percentage reduction in autoantibodies 85.6 ± 14.4% (P = 0.00014), and that in pemphigus disease area index was 75.4 ± 24.3% (P = 0.0023). No side-effects were observed. All patients exhibited clinical improvement after undergoing double-filtration plasmapheresis, and the prednisolone dose was reduced by 41 ± 8.9 mg (P = 0.0005) approximately 3 months after double-filtration plasmapheresis. To our knowledge, this is the first report evaluating the efficacy of double-filtration plasmapheresis with pemphigus disease area index, and it demonstrated that double-filtration plasmapheresis is a safe "subtracting" treatment for patients with drug-resistant pemphigus.

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confidence: 99%

Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

et al. 2017

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“…Though several case reports or series also reported the capability of plasmapheresis in inducing remission in severe or refractory pemphigus patients, 19,20,22,[30][31][32] high quality evidence was still lacking. In the American Society for Apheresis guidelines updated in 2019, plasmapheresis was recommended as category III (optimum role of apheresis therapy is not established), Grade 2B (weak recommendation, low-quality or very low-quality evidence) with the indication of severe cases.…”

Section: Discussionmentioning

confidence: 99%

“…17 Since then, there have been quite a few researches in this field, but the results are controversial. [18][19][20] The utilized techniques of plasmapheresis are also different in these studies. 21,22 Double filtration plasmapheresis (DFPP) is superior to other techniques, because it can increase the selectivity of the molecular size.…”

Section: Introductionmentioning

confidence: 99%

Double‐filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China

Liang

Zhang

et al. 2020

J of Clinical Apheresis

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Background: Pemphigus is a group of rare and severe autoimmune blistering disease mediated by pathogenic autoantibodies against desmogleins. Plasmapheresis can directly remove autoantibodies from circulation, which has been applied to the treatment of pemphigus as an adjuvant therapy. But the results of the researches are controversial. This study aims to evaluate the efficacy and safety of double filtration plasmapheresis (DFPP) combined with immunosuppressive treatment for patients with severe pemphigus in our single center. Methods: We retrospectively analyzed 17 patients with severe pemphigus who were unresponsive to high-dose corticosteroid and received DFPP treatment between January 2010 and January 2020. The information on demographic characteristics, clinical and laboratory data, treatment regimens, and clinical outcomes were collected. Results: All the patients were diagnosed as severe pemphigus and had a period of at least 1 week of high-dose prednisone (1-1.5 mg/kg/day), but they were unresponsive to corticosteroid and immunosuppressants treatment. They received DFPP treatment as an adjuvant therapy. After DFPP treatment, the titers of desmogleins antibodies significantly decreased (P < .001), Nikolsky's sign became negative and no new blisters appeared. The dosage of corticosteroid could begin to taper down rapidly in 9 ± 4 days. On discharge, the dosage of prednisone decreased significantly (51 ± 3 mg/day, P < .001). No major adverse events happened that could lead to the termination of DFPP treatment. Conclusion: Double filtration plasmapheresis combined with immunosuppressive treatment is an effective and safe therapeutic regimen for severe pemphigus. DFPP can also contribute to the dosage reduction of steroid to avoid more drug-related side effect.

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“…A tryptophan adsorber achieves only 30% reduction of pemphigus autoantibody and can significantly decrease fibrinogen levels . In a limited number of reports, DFPP was found to be useful in severe and refractory pemphigus vulgaris .…”

Section: Discussionmentioning

confidence: 99%

“…For patients with severe symptoms, those who received high doses of conventional agents, and/or those who have aggressive and rapidly progressive disease, the American Society for Apheresis recommends therapeutic plasma exchange and IA as Grades 2B and 2C, Category III . Moreover, the effectiveness of double filtration plasmapheresis (DFPP) in pemphigus vulgaris was reported .…”

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confidence: 99%

Selective Plasma Exchange for the Removal of Pemphigus Autoantibodies, Fibrinogen, and Factor XIII in Pemphigus Vulgaris

et al. 2017

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Pemphigus vulgaris is a serious autoimmune skin disorder associated with desmoglein 1 and 3. Selective plasma exchange (SePE) for pemphigus vulgaris remains unknown. We investigated the removal characteristics of pemphigus autoantibodies, immunoglobulins, and fibrinogen in three cases. When the mean processed volume for SePE was 1.2 plasma volumes, the mean percent reduction was 50.7% for desmoglein 1, 48.9% for desmoglein 3, 50.3% for IgG, 29.8% for IgA, 1.9% for IgM, and 17.6% for fibrinogen. In one case, the percent reduction after four sessions of SePE within eight days was 87.0% for desmoglein 1, 85.1% for desmoglein 3, 76.6% for IgG, 53.5% for IgA, 7.9% for IgM, 41.6% for fibrinogen, and 31.4% for factor XIII. SePE can effectively remove pemphigus autoantibodies and retain coagulation factors, e.g. factor XIII and fibrinogen. In severe cases, SePE can be useful and safe for induction therapy.

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Resistant oral mucosal lesions in pemphigus vulgaris responsive to double filtration plasmapheresis: First case report from Turkey

Cited by 5 publications

References 13 publications

Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

Evaluating the Efficacy of Double‐Filtration Plasmapheresis in Treating Five Patients With Drug‐Resistant Pemphigus

Double‐filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China

Selective Plasma Exchange for the Removal of Pemphigus Autoantibodies, Fibrinogen, and Factor XIII in Pemphigus Vulgaris

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