بررسی اطلاعات دموگرافیک و درمانی بیماران مبتلا به PV با remission کامل مراجعه کننده به بیمارستان رازی در سال های 85 تا 95

بررسی اطلاعات دموگرافیک و درمانی بیماران مبتلا به PV با remission کامل مراجعه کننده به بیمارستان رازی در سال های 85 تا 95

19 4 1397

حذف
تک ستونه
حذف
ویرایشگر متن
پمفیگوس ولگاریس یک بیماری اتوایمیون، داخل اپی تلیالی و تاولی است که بر پوست و غشاهای مخاطی اثر می کند. این بیماری با واسطه ی اتوآنتی بادی های درگردش علیه سطوح سلولی کراتینوسیتی ایجاد می شود. این بیماری بالقوه تهدیدکننده ی حیات است که میزان مرگ و میر تقریبی 5 تا 15 درصد دارد. پمفیگوس ولگاریس به طُرق مختلفی می تواند وارد فاز خاموشی شود که در آنها همه ی تظاهرات بیماری محو شده و همه ی درمان ها می توانند قطع شوند. چه مدت یکبار و چه مدت طول می کشد که خاموشی رخ دهد، به وضوح، روشن نیست. مرور مطالعات بزرگ PV انجام شده در طی چهار دهه ی گذشته نشان داده است که خاموشی در کمتر از یک سوم بیماران رخ می دهد. اما مشکل این مطالعات این است که بروز خاموشی معمولاً فقط در یک نقطه ی زمانی اندازه گرفته شده است. بنابراین، روشن نیست که چه مدت طول می کشد تا خاموشی ایجاد شود، چه مدت خاموشی طول می کشد و وقتی که درمان قطع شود، چه رخ می دهد. با توجه به این مساله، ما در این مطالعه ما بر آن شدیم که اطلاعات دموگرافیک و درمانی بیماران مبتلا به  PV  با remission کامل، مراجعه کننده به بیمارستان رازی،  در فاصله ی ده ساله ی سال 1385 تا 1395 را مورد بررسی قرار دهیم.
مواد و روش‌ها:
در این مطالعه که به صورت گذشته نگر در بیمارستان رازی تهران انجام شد،  پرونده های درمانگاه پمفیگوس بین سالهای 85 تا 95 انتخاب خواهد شد. پرونده ها بررسی شده و مواردی که فقدان هیچ ضایعه ی جدید یا مستقر ایجاد شده و در حالی که بیمار هیچ گونه درمان سیستمیک برای حداقل دو ماه نداشته باشد، وارد مطالعه شدند. پژوهشگر آموزش دیده، داده های موردنیاز مطالعه را از پرونده های استخراج نموده و وارد فرم های از پیش آماده کرد. داده های در صورت نقصان، با پیگیری تلفنی تکمیل گردید. سپس داده های وارد نرم افزار SPSS نسخه 25 خواهد شد و مورد آنالیز توصیفی، کمی، کیفی و تحلیلی قرار گرفت.
نتایج:
در مطالعه ی ما 55 بیمار که پرونده­های آنها با دو معیار از سه معیار پاتولوژی یا DIF  یا AntiDsg مبنی بر بیماری پمفیگوس وولگاریس یا فولیاسه یا پارانئوپلاستیک و سابقه ی حداقل یک نوبت خاموشی کامل تا اول اردیبهشت ماه 97 وجود داشتند، از بین سایر بیماران جدا شده و تحت بررسی قرار گرفتند. 21 بیمار (38%) مرد و 34 بیمار (62%) زن بودند که این آمار با شیوع و فراوانی این بیماری در اطلاعات دموگرافی در سایر مطالعات همخوانی داشت. اطلاعات درمانی شامل طول دوره ی بیماری، طول دوره ی خاموشی و دارو های دریافتی و اطلاعات پاراکلینیکی بیماران مورد بررسی و مقایسه قرار گرفت و وجود ارتباط معنی دار یا عدم وجود آن مورد ارزیابی قرار گرفت.
نتیجه گیری:
اطلاعات دموگرافیک و درمانی در بیماران پمفیگوس مورد بررسی در این مطالعه نشان میدهد به طور کلی ارتباط معنی داری بین پروتوکل های درمانی بیماران و طول مدت بیماری و خاموشی در بیماران وجود دارد و دریافت ریتاکسی مب چه در مراحل اولیه ی بیماری چه در موارد پیشرفته یا گسترده ی بیماری نتایج بالینی و پاراکلینیکی خوبی به همراه داشته است.
کلمات کلیدی:
پمفیگوس – خاموشی – عود – ریتاکسی مب – AntiDsg1  - AntiDsg3DIF -
مشخصات دانشجو:
نام: امیرحسین جدیدیان      
رشته تحصيلي: پزشکی
مقطع: عمومی                   گروه آموزشي: پوست
پست الكترونيك دانشجو: jadidian.amirhossein@gmail.com
اساتيد راهنما و داور:
استاد راهنما: دکتر حمیدرضا محمودی
اساتيد مشاور: دکتر بلیغی- دکتر دانش پژوه
اساتيد داور: دکتر عزیزپور- دکتر قندی
زمان دفاع :
   روز سه شنبه  تاريخ  19 تیر 1397 ساعت 12:30 ظهر
مكان دفاع به آدرس:
میدان وحدت- بیمارستان رازی- سالن آمفی تئاتر
اطلاعات به زبان انگليسي
Title:
Evaluation of demographic and therapeutic data in patients with PV with complete remission in Razi Hospital :referring from 1385 to 1395
 
Abstract:
Background and aim:
Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes. It is mediated by circulating autoantibodies directed against keratinocyte cell surfaces. A potentially life-threatening disease, it has a mortality rate of approximately 5-15%.
The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base (see the image below).

Signs and symptoms

Mucous membranes
Mucous membranes of the oral cavity are involved in almost all patients with pemphigus vulgaris.
Patients may have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal.
Intact bullae are rare in the mouth.
Erosions may be seen on any part of the oral cavity, and they may spread to involve the larynx, with subsequent hoarseness.
In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of cases.
Other mucosal surfaces may be involved, including the conjunctiva, esophagus (causes odynophagia and/or dysphagia), labia, vagina, cervix, vulva,  penis, urethra, nasal mucosa, and anus.
Skin
Primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base.
Blisters are fragile and may rupture, producing painful erosions (the most common skin presentation).
Nails
Acute or chronic paronychia, subungual hematomas, and nail dystrophies affecting one or several fingers or toes have been reported with pemphigus vulgaris.
Vegetating pemphigus vulgaris
Lesions in skin folds readily form vegetating granulations. In some patients, erosions tend to develop excessive granulation tissue and crusting; these individuals display more vegetating lesions.

Diagnosis

Laboratory studies include the following:
  • Histopathology: Demonstrates an intradermal blister; the earliest changes consist of intercellular edema with loss of intercellular attachments in the basal layer
  • Direct immunofluorescence (DIF): On normal-appearing perilesional skin; demonstrates in vivo deposits of antibodies and other immunoreactants, such as complement
  • Indirect immunofluorescence (IDIF): If DIF results are positive; circulating intercellular antibodies are detected using IDIF in 80-90% of patients with pemphigus vulgaris

Management

The aim of pharmacologic therapy for pemphigus vulgaris is to reduce inflammatory response and autoantibody production. Medications used in the disease’s treatment include the following:
  • Corticosteroids: Discourage the inflammatory process by inhibiting specific cytokine production
  • Immunosuppressants: Should be considered early in the course of disease as steroid-sparing agents
 
 In Iran, there is no study about this rare dermatologic disease. Razi hospital as a referral center of dermatologic disease in country, has a good potential of data gathering and analysis of LABD in Iran.
 
Methods and Materials:
This retrospective study was conducted on patients with positive direct immunofluorescence microscopy or relative pathology or positive AntiDsg ith at least one remission referred to Razi hospital between 2006 to 2016. Medical records of all patients with above characteristics were reviewed and according to prepared questionnaires, we have gathered demographics data such as age, sex, past medical history, drug history and so on. Treatments, remission time period and disease duration also have been reviewed.
Data were analyzed with SPSS 25.
 
Results:
In our study, 55 patients with dual criteria of either pathology or DIF or AntiDsg based on Pemphigus Vulgaris, or Foliaceus or paraneoplastic disease, with a history of at least one complete remission until May 1, 97, were examined. 21 patients (38%) were male and 34 patients (62%) were women. This finding was consistent with the incidence and frequency of the disease in demographic information in other studies. Therapeutic information including the duration of the disease, the remissions periods and received drugs, and paraclinical information of the patients were evaluated and compared then reported if there was a significant correlation.
Conclusion:
Demographic and therapeutic data in Pemphigus patients in this study indicate that there is a significant relationship between patients' treatment protocols and duration of disease and remission in patients and receiving rituximab in the early stages of the disease, whether in advanced or the widespread illness has had good clinical and paraclinical results.
 
 
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