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"Penatalaksanaan kasus pemfigus vulgaris pada pasien lansia dengan riwayat penyakit kronis. Pemfigus vulgaris (PV) adalah sekelompok penyakit vesikulobulosa yang dapat mengenai kulit dan membran mukosa. PV merupakan penyakit autoimun dengan karakteristik adanya ikatan antara autoantibodi IgG dengan desmoglein 3 pada desmosom. Lesi PV merupakan bula berdinding tipis yang timbul pada kulit dan mukosa yang terlihat normal. Bula pada mukosa oral sangat mudah pecah dan dan membentuk ulser. Terdapat beberapa faktor yang dapat mencetuskan PV meliputi faktor genetik, usia, pemakaian obat-obatan dan makanan. Tulisan ini melaporkan penatalaksanaan kasus yang diduga adalah PV yang terjadi pada lansia dengan riwayat penyakit kronis. Kasus adalah wanita berusia 75 tahun dengan riwayat adanya lesi bula di kulit sejak 2 bulan dan ulserasi oral yang menetap sejak 1 bulan yang lalu. Ulserasi terlihat ditutupi oleh lapisan fibrin dan erosif eritema pada mukosa labial, bukal, dan gingiva. Terdapat riwayat hipertensi, diabetes mellitus, hepatitis, dan trombositopenia. Diagnosis PV ditegakkan berdasarkan keluhan subjektif dan klinis yang dapat dibedakan dengan penyakit vesikobulosa yang lain. Kortikosteroid sistemik diberikan bersama hepato protektor, obat kumur, terapi suportif, dan krim bibir. Bula pada kulit dan ulserasi yang menetap pada mukosa oral adalah manivestasi PV. Pada pasien lansia ini keadaan sistemiknya membutuhkan penatalaksanaan yang lebih intensif dan komprehensif.

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Pemphigus vulgaris (PV) is a group of vesicobullous disease that affects skin and mucous membranes. PV is associated with autoimmune diseases, characterized by binding of IgG autoantibodies to desmoglein 3 on desmosome. The lesion of PV is a thin-walled bulla arising on the normal skin or mucosa. The bulla of oral mucous are more fragile and rapidly break down, forming irreguler ulcers. There are several factors that can trigger PV including genetic factors, age, drugs and food. The purpose of this paper was to report the treatment of suspected pemphigus vulgaris in elderly patient with history of chronic diseases. A 75-year-old woman with 2 months history of bulla formation on skin and about a month history of persistent oral ulceration. Ulcers were covered with fibrin and erosive erythematous on labial mucosa, buccal mucosa and gingival. The patient has a story of hypertension, diabetes mellitus and hepatitis. On routine complete blood count was found thrombocytopenia. Diagnosis was confirmed by anamnesis, clinical examination and complete blood investigation. It should be distinguished from other vesicobullous disease. Systemic corticosteroid therapy was given concurrently with hepato-protector, mouthwash, supportive therapy and topical lip cream. Bulla on skin and persistent erosive ulcers of the oral mucosa are the manifestations of PV. Elderly patient with history of chronic diseases is a complex care of PV and this require more intensive and comprehensive treatment."

"Pemphigus vulgaris is a relatively uncommon autoimmune disease marked with blister appearance of the skin tissue and mucosal membrane, resulting in loss of intercellular adhesion as well as the intact of epithelial cells, or so-called acantholysis. In this autoimmune disease the immune system forms autoantibodies attacking desmoglein, a specific protein at the skin tissue. The predisposing factors are presumed to include genetic and ethnic origin of the patient. Although at first usually without symptoms, after proceeding to the ulcerating stage the disease can result in fatal complications. Considering that almost 60% of the cases are first manifested in the oral mucosa, it is important that the dentists are aware of the basic etiology and immunology of the disease for correct diagnosis and therapy. This work reviews the etiopathogenesis of Pemphigus vulgaris, with emphasis on immunological aspects and immunotherapy."

"Pemphigus vulgaris is an autoimmune–mediated disease of skin and mucous membran leading to progressive blistering and chronic erosions. It often begins with blister formations which easily rupture. The characteristic feature is positive nikolsky sign which may or not be presented simultaneously. Infrequently, ocular involvement may be seen as conjunctivitis. Establishment of early definite diagnosis is critical and requires correlation of clinical and histopathological findings. Because of this conditon is a potentially life-threatening, the risk of complications and mortality rate increases if initial management is non comprehensive and inadequate. Treatment is directed at supression of autoimmune process, typically administration of corticosteroids. This article report a case in a 51 years old woman who had painful chronic oral ulcer and poor general health condition. Prior to the visit to Oral Medicine clinic, patient was treated by her general practitioners for several months, without either established diagnosis nor comprehensive and adequate management, so that she had no clinical improvement. Clinical examination at the first visit in OralMedicine clinic Cipto Mangunkusumo Hospital, revealed bula in the skin, conjunctivitis, easily bleed oral mucosae, widespread erosions and ulcerations of the lips, gingiva, tongue, and buccal mucosae. Biopsy of one of skin erosions demonstarting suprabasal intraepithelial acantholysis. Multi divisions in Cipto Mangunkusumo Hospital, such as Oral Medicine, Dermatology, Internal Medicine, Ophtalmology, ENT, were involved in treating this case. Patient received high dose methylprednisolone sistemically and prednisone topically for the lips. General remission achieved in several days. Thus, establishment of early definite diagnosis and adequate management are important in management of Pemphigus vulgaris."

"Background: autoimmune patients can be more susceptible to infection. Proper knowledge, perception, and practices towards COVID-19 are essential for these patients during pandemic. This study aimed to know their knowledge, perception, and practices regarding COVID-19. Methods: cross sectional study using online survey was conducted from April to May 2020. Patients with autoimmune disease were asked about demographic characteristics, diagnosis, history of treatment, knowledge, perception, and practice regarding COVID-19. Results: there were 685 respondents. Most of them were female and had systemic lupus erythematosus with median age of 37 years old. Almost all respondents had good knowledge regarding transmission of COVID-19 and did proper prevention practices. Adequacy of information and steroid or mycophenolate mofetil/mycophenolic acid (MMF/MPA) use were related to perception of the effect of pandemic to their own health. Visiting private clinic and receiving hydroxychloroquine/chloroquine sulfate or sulfasalazine were related to perception that autoimmune conditions would make them more prone to COVID-19. Work from home was related to perception that when contracting COVID-19, the symptoms would be more severe. Living in Sumatra region and getting hydroxychloroquine/ chloroquine sulfate or MMF/MPA were related to perception that autoimmune medications could reduce risk of getting COVID-19. Adequate information, university education, private clinic visit, and hydroxychloroquine/ chloroquine sulfate use were related to perception that COVID-19 pandemic would cause difficulties in getting medications. Conclusion: almost all respondents had good knowledge and practices regarding COVID-19. Adequacy of information, autoimmune treatment, work from home, educational background, area of living, and health care facilities contributed to perception regarding COVID-19 pandemic."

"ABSTRAKSystemic Lupus Erythematosus SLE adalah penyakit autoimun, dimana sistem kekebalan tubuh menyerang organ tubuh sendiri. Orang dengan Lupus Odapus mengalami berbagai perubahan secara fisik, ekonomi, sosial, dan psikologis yang menyebabkan mereka memiliki penghayatan akan penyakit yang dimiliki, atau disebut juga illness cognition. Penghayatan ini mempengaruhi kognisi Odapus dalam memandang penyakit dan mempengaruhi perilaku kesehatan serta coping akan permasalahan yang disebabkan oleh SLE. Penelitian bertujuan untuk mengubah illness cognition Odapus terkait penyakit SLE yang dimiliki. Penelitian merupakan penelitian kuasi-eksperimental dengan one group pretest-posttest design. Kelompok terdiri dari lima orang yang diperoleh lewat accidental sampling. Partisipan mengikuti lima kali sesi individual serta satu kali pra-sesi dan satu kali sesi follow-up. Analisis dilakukan dengan cara membandingkan data kuantitatif menggunakan adaptasi alat ukur Illness Cognition Questionnaire ICQ serta data kualitatif tentang perubahan kognitif, perilaku, dan strategi pemecahan masalah sebelum dan sesudah mengikuti intervensi. Hasil penelitian menunjukkan bahwa intervensi kognitif-perilaku dapat meningkatkan illness cognition pada Odapus. Partisipan dapat menerima penyakit SLE sebagai bagian dari hidupnya, menghayati adanya berbagai hal positif dari penyakit SLE, dan memiliki harapan serta dapat melakukan kontrol terhadap berbagai keterbatasan yang diakibatkan oleh penyakit SLE. Pada akhirnya, partisipan dapat meningkatkan kemampuan untuk menyelesaikan masalah yang diakibatkan oleh penyakit SLE secara lebih efektif.

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ABSTRACTSystemic Lupus Erythematosus SLE is autoimmune disease which the immune system damage their own body. Patient with SLE experience change in physical, economic, social, and psychology that caused a perception to their own disease, called illness cognition. This perception influence patient rsquo s cognition about their disease and predispose their health behavior and coping problem related to their disease. This study aimed to identify effectiveness of cognitive behavior therapy to change illness cognition patient about SLE. This was quasi experimental study conducted with one group pre test post test design. Group consisted of five participant recruited through accidental sampling. Participants participated in five individual sessions, preceded by a pre session and followed by a follow up session. Analysis was conducted by comparing quantitative data obtained by Indonesian adaptation of Illness Cognition Questionnaire ICQ and qualitative data showing changes in participants rsquo cognition, behavior, and problem solving before and after the intervention took place. This study showed that cognitive behavior therapy can successfully enhance illness cognition in patient with SLE. Participants may receive SLE disease as a part of their life, appreciate many positive things of SLE, have hope also can control various limitations caused by SLE. Participants can improve their ability to coping with problem related to SLE more effectively."

"LES merupakan penyakit inflamasi autoimun kronis dan banyak terjadi pada  anak remaja dengan rata-rata onset usia 11-12 tahun. Sekitar 10% dari remaja dengan penyakit kronis seperti LES mengalami masalah psikososial, termasuk masalah emosi seperti depresi dan kecemasan. Penelitian ini bertujuan untuk mengetahui apakah pelatihan kecakapan hidup pada anak dengan LES dapat memperbaiki masalah emosi. Penelitian dilakukan dengan 30 subjek remaja perempuan dengan LES yang sudah mendapatkan pengobatan, dan nilai SLEDAI 0-5. Subjek dibagi menjadi 2 kelompok secara acak tanpa penyamaran, perlakuan dan kontrol.  Pelatihan kecakapan hidup diberikan pada kelompok perlakuan sebanyak 1 kali dalam kelas. Perbaikan masalah emosi dinilai dengan membandingkan nilai SDQ sebelum pelatihan dan 4 minggu setelah pelatihan. Penelitian melibatkan 30 remaja perempuan dengan LES dengan usia rerata 14 tahun. Sebanyak 20/30 subjek memiliki nilai SDQ normal, 4/30 dengan SDQ borderline dan 6/30 dengan SDQ abnormal. Terdapat perbedaan bermakna selisih masalah emosi pada kedua kelompok (p: 0,025; effect size: 0,87). Pada kelompok yang mendapatkan pelatihan terdapat perbaikan nilai SDQ total (p: 0,001), nilai masalah emosi (p: 0,002), nilai masalah perilaku (p: 0,027) dan nilai masalah perilaku hiperaktif (p: 0,040) dibandingkan dengan awal studi. Sedangkan pada kelompok kontrol hanya terdapat perubahan nilai masalah dengan teman sebaya (p: 0,011). Selain itu ditemukan pula perbaikan masalah emosi pada kelompok pelatihan yakni keluhan sakit fisik (p: 0,021), rasa khawatir (p: 0,020) dan perasaan gugup (p: 0,020). Studi ini menyimpulkan bahwa pelatihan kecakapan hidup-modul pengelolaan emosi efektif dalam memperbaiki masalah emosi pada remaja perempuan dengan LES secara signifikan, terutama gugup atau sulit berpisah dengan orangtua/pengasuhnya pada situasi baru, mudah kehilangan rasa percaya diri dan banyak kekhawatiran atau sering tampak khawatir.

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SLE is a chronic autoimmune inflammatory disease and many occur in adolescents with an average age of onset of 11-12 years. About 10% of adolescents with chronic diseases such as SLE experience psycho-mental problems, including emotional problems such as depression and anxiety. The aim of this study is to determine whether life skills training in children with SLE can improve emotional problems. The study was conducted with 30 female adolescent with SLE who had received treatment and SLEDAI score 0-5. Subjects were divided into 2 groups randomly, not-blinding, experiment and control. Life skills training is given to the experiment group one time in group. Emotional problem improvement was assessed by comparing SDQ scores before training and 4 weeks after training. The study involves a total of 30 female adolescent with SLE with an average age of 14 years. A total of 20/30 subjects had normal SDQ values, 4/30 with borderline SDQ and 6/30 with abnormal SDQ. There were significant differences in the difference between emotional problems in the two groups (p: 0.025; effect size: 0.87). In the group that received training there was an improvement in the total SDQ value (p: 0.001), the value of emotional problems (p: 0.002), the value of conductive problems (p: 0.027) and the value of hyperactive behavior problems (p: 0.040) compared to the beginning of the study. Whereas in the control group there were only changes in the value of problems with peers (p: 0.011). In addition it also found improvements in emotional problems in the experiment group, they are complaints of physical pain (p: 0.021), anxiety (p: 0.020) and nervous feelings (p: 0.020). This study concludes that life skills training-emotion management module is significantly effective in improving emotional problems in female adolescent with LES, especially nervous or having difficulty separating from parents/caregivers in new situations, easily losing self-confidence and many worries or often seems worried."

"ABSTRAKLatar belakang. Multipel Sklerosis MS dan Neuromyelitis Optica Spectrum Disorder NMOSD adalah penyakit autoimun yang mengakibatkan lesi inflamasi dan demielinisasi pada sistem saraf pusat. Salah satu manifestasi klinis yang paling menonjol pada kedua penyakit ini adalah neuritis optik NO . Penelitian ini bertujuan untuk mengetahui perbedaan struktur dan fungsi nervus optikus pada pasien MS dan NMOSD serta melihat hubungannya dengan derajat disabilitas yang dinilai dengan EDSS, durasi penyakit, dan jumlah relaps. Metode. Penelitian ini menggunakan desain potong lintang dengan jumlah sampel 30 mata MS dan 15 mata NMOSD di Rumah Sakit Cipto Mangunkusumo RSCM , tanpa riwayat neuritis optik dalam 6 bulan terakhir. Penelitian dilakukan pada bulan Desember 2017. Struktur nervus optikus dinilai dengan menggunakan optical coherence tomography yang menilai ketebalan GCL-IPL ganglion cell layer-inner plexiform layer dan RNFL retinal nerve fiber layer serta foto fundus. Sedangkan fungsi nervus optikus dinilai dengan ketajaman penglihatan logmar , sensitivitas kontras, dan latensi P100. Hasil. Rerata usia MS 30 6 tahun tahun dan NMOSD 23,19 7,25 tahun. Hanya terdapat satu orang laki-laki pada kelompok MS, sedangkan subjek NMOSD keseluruhannya adalah perempuan. Kelompok NMOSD memiliki ketebalan lapisan GCL-IPL, RNFL serta ketajaman penglihatan yang lebih rendah dibandingkan MS. Pada kelompok ini juga didapatkan korelasi positif antara nilai EDSS dengan ketajaman penglihatan r=0,74 dan korelasi negatif dengan rerata ketebalan lapisan GCL-IPL r=-0,67 dan RNFL r=-0,46 . Pada kelompok MS, subjek dengan nilai EDSS yang tinggi cenderung memiliki lapisan GCL-IPL yang lebih tipis. Korelasi negatif antara durasi penyakit dengan ketajaman penglihatan r=0,65 dan ketebalan lapisan GCL-IPL r=-0,63 terlihat pada kelompok NMOSD. Sedangkan pada MS didapatkan korelasi negatif antara durasi penyakit dengan sensitivitas kontras r=-0,42 serta ketebalan lapisan GCL-IPL r=-0,40 dan RNFL r=-0,38 . Jumlah relaps berkorelasi negatif dengan ketebalan lapisan RNFL r=-0,63 pada kelompok NMOSD. Pada kelompok MS, jumlah relaps tidak berkorelasi dengan parameter struktur dan fungsi nervus optikus. Kesimpulan. Ketebalan lapisan GCL-IPL dan RNFL lebih tipis pada kelompok NMOSD. Fungsi nervus optikus pada NMOSD juga lebih inferior dibandingkan MS. Derajat disabilitas dan durasi penyakit berkorelasi dengan parameter struktur dan fungsi nervus optikus pada pasien MS dan NMOSD. Sedangkan korelasi dengan jumlah relaps hanya didapatkan pada kelompok NMOSD.

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ABSTRACT<>br>Background. Multiple Sclerosis MS and Neuromyelitis Optica Spectrum Disorder NMOSD is an autoimmune disease that results in inflammatory lesions and demyelinization of the central nervous system. One of the most prominent clinical manifestations in both diseases is optic neuritis ON . This study aims to determine the differences in structure and function of the optic nerve in MS and NMOSD patients and to see its relationship with the degree of disability assessed by EDSS, duration of disease, and number of relapse. Method. This study used cross sectional design with 30 MS eyes 15 NMOSD eyes at Cipto Mangunkusumo Hospital RSCM , with no history of optic neuritis in the last 6 months. The study was conducted in December 2017. The optic nerve structure was assessed using optical coherence tomography by measuring the thickness of GCL IPL ganglion cell layer inner plexiform layer and RNFL retinal nerve fiber layer and fundus photography. While optic nerve function is assessed with visual acuity, contrast sensitivity, and P100 latency. Results. Mean age of MS subjects were 30 6 years and NMOSD 23.19 7.25 years. There is only one male in the MS group, while the entire NMOSD subject is female. The NMOSD group has lower GCL IPL and RNFL thickness and also lower visual acuity than MS. In this group there was also a positive correlation between EDSS value with visual acuity r 0.74 and negative correlation with mean GCL IPL r 0.67 and RNFL thickness r 0.46 . In the MS group, subjects with high EDSS values tend to have thinner GCL IPL. The positive correlation between disease duration and visual acuity r 0.65 and negative correlation with GCL IPL layer thickness r 0.63 was seen in the NMOSD group. While in MS, there was a negative correlation between duration of disease with contrast sensitivity r 0.42 and mean GCL IPL r 0.40 and RNFL thickness r 0.38 . The number of relapse were negatively correlated with mean RNFL thickness r 0.63 in the NMOSD group. In the MS group, the number of relapse was not correlated with the structural and functional parameters of the optic nerve. Conclusion. The thickness of the GCL IPL and RNFL is thinner in the NMOSD group. The optic nerve function in NMOSD is also inferior to MS. The degree of disability and duration of disease correlates with the structural and functional parameters of the optic nerve in MS and NMOSD patients. While the correlation with the number of relapse is only found in the NMOSD group."

"Latar belakang: Artritis idiopatik juvenil (AIJ) adalah penyakit autoimun yang ditandai dengan peradangan sendi kronis. Anak dengan AIJ akan mengalami hambatan pertumbuhan tulang yang disebabkan beberapa mekanisme langsung maupun tidak langsung. Sebanyak 40-50 % pasien AIJ memiliki densitas mineral tulang yang rendah pada tulang belakang lumbal dan panggul. Densitas mineral tulang yang rendah dipengaruhi beberapa faktor yaitu klasifikasi penyakit, lama sakit, indeks masa tubuh, status pubertas, aktivitas penyakit, aktivitas fisik, kadar 25(OH)D, dosis kumulatif kortikosteroid, dan dosis metotreksat.Tujuan: Penelitian ini dilakukan untuk mengetahui densitas mineral tulang pada pasien AIJ dan faktor-faktor yang berhubungan.Metode: Penelitian ini merupakan studi potong lintang dengan melibatkan 32 pasien AIJ. Pemilihan subjek dilakukan berdasarkan data registri pasien AIJ di poliklinik Alergi-Imunologi RSCM dan RSAB Harapan Kita tahun 2014-2019. Densitas mineral tulang diperiksa dengan Dual X-ray Absorbtiometry (DEXA) dengan melihat skor Z. Dilakukan analisis bivariat untuk mencari hubungan antara variabel terhadap densitas mineral tulang.Hasil: Densitas mineral tulang total rerata adalah 0,86 g/cm2. Sebanyak 22 subjek mempunyai densitas mineral tulang rendah (osteopenia) dengan nilai skor-Z L1-L4 ≤-2 sedangkan 10 subjek menunjukkan hasil normal. Tidak ditemukan fraktur tulang belakang pada seluruh subjek. Osteopenia banyak ditemukan pada anak dengan dosis kumulatif metotreksat yang lebih banyak (p=0,016). Faktor-faktor lainnya tidak terbukti berhubungan dengan densitas mineral tulang yang rendah.Simpulan: Sebagian besar pasien AIJ mengalami gangguan densitas mineral tulang. Dosis metotreksat yang dihubungkan dengan aktivitas penyakit merupakan faktor yang berperan untuk terjadinya osteopenia.

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Background: Juvenile Idiopathic Arthritis (JIA) is an autoimmune disease characterized by chronic inflammatory arthritis. The disease will affect bone development in children with JIA through direct and indirect mechanisms. About 40-50 % patient with JIA have low bone mineral density in the spine. The low bone mineral density is associated with disease classification, disease duration, body mass index, puberty status, disease activity, physical activity, 25(OH)D level, cumulative doses of corticosteroid and methotrexate.

Objective: This study aimed to investigate bone mineral density in children with JIA and its associated factors.

Method: A cross-sectional study involving 32 children with JIA. Patients were selected based on registry data in the outpatient clinic, subdivision of Allergy and Immunology, Department of Child Health, Dr. Cipto Mangunkusumo General Hospital and Harapan Kita Women and Children Hospital between 2014-2019. Bone mineral density was measured using Dual X-ray Absorbtiometry (DEXA) and reported using Z score. Bivariate analysis was used to identify factors associated with bone mineral density.

Result: The mean bone mineral density was 0,86 g/cm2. Low bone mineral density (osteopenia) occurred among 22 patients (Z score ≤-2 at L1-L4). 10 patients have normal bone mineral density. No vertebral fracture was seen on x-ray. Osteopenia was mainly found in patients with higher cumulative doses of methotrexate (p=0,016). The other factors were not associated with low bone mineral density.

Conclusion: Most patients with JIA have low bone mineral density. Low bone mineral density tends to occur among patients with higher cumulative doses of methotrexate treatment."