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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."

"Dermatosis autoimun bulosa (DAB) adalah sekumpulan penyakit kronik autoimun dengan ciri lepuh dan lecet pada kulit dan/atau mukosa; termasuk di dalamnya adalah pemfigus dan pemfigoid. Gangguan psikologis yang berat, hingga misalnya keinginan bunuh diri, terkadang dijumpai. Tetapi, depresi pada DAB belum banyak diteliti. Sebuah studi potong lintang observasional-analitik dilakukan di sebuah rumah sakit rujukan tersier di Jakarta pada Desember 2020-Maret 2021 untuk mengetahui prevalensi depresi pada pasien DAB serta faktor sosiodemografi dan klinis yang berhubungan. Sejumlah 33 orang subjek berusia minimal 18 tahun yang terdiagnosis DAB, tidak sedang remisi, tanpa riwayat depresi sebelum diagnosis tersebut ataupun gangguan psikiatrik lainnya, mengikuti penelitian. Patient Health Questionnaire-9 (PHQ-9) versi Bahasa Indonesia yang telah divalidasi digunakan untuk menapis depresi dengan nilai cut-off ≥10. Beberapa variabel sosiodemografi dan klinis, termasuk tingkat risiko stres berdasarkan life events (Skala Stres Holmes dan Rahe), serta keparahan penyakit berdasarkan Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) diidentifikasi. Sebagian besar subjek adalah perempuan (69,7%), berusia 47,36±13,5 tahun, menikah (78,8%), tingkat pendidikan menengah (57,6%), tidak bekerja (57,6%), penghasilan rendah (60,7%), tidak memiliki riwayat depresi pada keluarga (100%), tingkat risiko stressful life events rendah (63,6%), terdiagnosis pemfigus vulgaris (60,6%), lama sakit 1-5 tahun (72,7%), median skor ABSIS 8,75, tanpa lesi mukosa (54,5%), bergejala terkait DAB (60,6%), keterlibatan lokasi terbuka (69,7%), disertai komorbid (78,8%), menggunakan kortikosteroid sistemik ≥4 minggu (78,8%) dengan rerata dosis harian <40mg/hari (87,9%), serta mendapat juga imunosupresan lain (66,7%). Prevalensi depresi pada pasien DAB adalah 24,2%, sedangkan pada pemfigus vulgaris sebesar 40%. Berdasarkan uji bivariat, terdapat hubungan depresi dengan tingkat pendidikan (p=0,082), tingkat stressful life events (p=0,015), diagnosis pemfigus vulgaris (p=0,012), dan keterlibatan lokasi terbuka (p=0,071). Analisis multivariat mendapatkan peningkatan risiko depresi pada tingkat pendidikan tinggi (adjusted OR 9,765; p=0,039), serta skor ABSIS yang lebih tinggi daripada 1 angka di bawahnya (adjusted OR 1,039; p=0,038). Prevalensi depresi pada DAB lebih tinggi daripada di populasi umum Indonesia. Penapisan disarankan khususnya pada pasien pemfigus vulgaris, berpendidikan tinggi, dan/atau dengan kondisi yang parah. Penelitian lanjutan diperlukan untuk memastikan temuan studi pendahuluan ini.

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Autoimmune bullous diseases (AIBD) is a group of chronic autoimmune dermatoses characterized by blisters and sores on the skin and/or mucosa; among them are pemphigus and pemphigoids. Severe psychological problems, even leading to suicidal thought, are not uncommonly encountered. However, depression in AIBD is rarely studied. A cross-sectional, observational analytical study was conducted in a tertiary referral hospital in Jakarta from December 2020 through March 2021 to determine the prevalence of depression among AIBD patients and related sociodemographic and clinical characteristics. Thirty-three AIBD subjects aged 18 years or older, not in remission, without recorded depression prior to diagnosis or other psychiatric disorders, were recruited. A validated Patient Health Questionnaire-9 (PHQ-9) in Indonesian language was used to screen depression with cut-off score ≥10. Several sociodemographic and clinical characteristics, including stress-risk level according to life events by Holmes and Rahe Scale and disease severity by Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) were identified. Majority of subjects were women (69.7%), aged 47.36±13.5 year-old, married (78.8%), had middle-level education (57.6%), unemployed (57.6%), low income (60.7%), without family history of depression (100%), experiencing low-risk stressful life events (63.6%), diagnosed with pemphigus vulgaris (60.6%), disease duration 1-5 years (72.7%), median of ABSIS score 8.75, without mucosal lesion (54.5%), suffering from symptoms related to AIBD (60.6%), showing involvement of exposed areas (69.7%), with comorbidities (78.8%), treated with systemic corticosteroids ≥4 weeks (78.8%) with daily doses <40mg/day (87.9%), and receiving also other immunosuppressive agents (66.7%). Prevalence of depression was 24.2% among AIBD and 40% among pemphigus vulgaris patients. Bivariate analysis showed significant correlation between depression and education level (p=0.082), stressful life events score (p=0.015), diagnosis of pemphigus vulgaris (p=0.012), and involvement of exposed areas (p=0.071). Multivariate analysis showed increased risk of depression at high level of education (adjusted OR 9.765; p=0.039) and ABSIS score higher than 1 point below (adjusted OR 1.039; p=0.038). Prevalence of depression among AIBD patients was higher than that among Indonesia’s general population. Screening is advised especially among those with pemphigus vulgaris, high level of education and/or severe condition. Further study is needed to confirm these early findings."

"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."

"Systemic lupus erythematosus (SLE) is a chronic excacerbative autoimmune disease with wide clinical spectrum. Gastrointestinal manifestasion is a frequent clinical manifestasion seen in SLE. Management with glucocorticoid and non-steroid anti-inflammatory drugs (NSAID) can mask the gastrointestinal symptoms in patient with SLE. One of the etiologies of gastrointestinal manifestations in SLE is acute appendicitis. Patients with acute appendicitis usually have abdominal pain as its chief complaint. The pathophysiology of acute appendicitis can occur primarily from SLE and secondary from other causes eg: infection, inflammation, etc. When a SLE patient has acute appendicitis as its initial assessment, determining its etiology is pivotal to give comprehensive management and preventing life-threatening complications.

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Lupus eritematosus sistemik (LES) adalah suatu penyakit autoimun kronis eksaserbatif dengan manifestasi klinis yang sangat beragam. Manifestasi gastrointestinal merupakan manifestasi yang sering dijumpai namun dapat terjadi efek masking oleh karena penggunaan obat-obatan untuk mengontrol penyakitnya seperti obat anti-inflamasi non-steroid (OAINS) dan kortikosteroid. Appendisitis akut merupakan salah satu penyebab nyeri abdomen pada penderita LES. Patofisiologi appendisitis akut dapat terjadi primer oleh aktivitas penyakitnya maupun sekunder oleh sebab lain. Membedakan etiologi appendisitis akut perlu dilakukan untuk memberikan tatalaksana yang komprehensif pada penderita dengan LES."

"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."

"Latar Belakang: Penelitian ini bertujuan membahas hubungan antara tingkat kemandirian pasien geriatri dan beban pramurawat yang merawatnya. Metode: Penelitian ini merupakan studi potong lintang, dengan cara pengambilan sampel consecutive sampling, dengan subyek sebanyak 116 yang merupakan pramurawat pasien geriatri yang menjalani rawat jalan pada bulan Juli 2013-Agustus 2013. Seluruh subyek diminta mengisi lembar kuesioner ZBI, serta instrumen ADL yang diisi oleh peneliti, kemudian dilakukan analisis terhadap data yang sudah terkumpul. Hasil: Gambaran kemandirian pasien geriatri diukur menggunakan intrumen Activities of Daily Living (ADL), Pada hasil didapatkan rerata skor ADL adalah 13 (SD 16, min-maks 0-20). Didapatkan gambaran beban pramurawat yang diukur dengan menggunakan instrumen The Zarith Burden Interview (ZBI) didapatkan rerata skor beban adalah 29,2 (10-61). Terdapat korelasi yang bermakna antara skor ADL dengan skor beban dengan r= -0,38 dengan p < 0,001. Kesimpulan: Terdapat hubungan yang bersifat terbalik antara tingkat kemandirian pasien geriatri dengan beban pramurawat yang merawatnya. dengan kekuatan korelasi lemah. Perlu dilakukan upaya untuk meningkatkan kemandirian pasien geriatri serta dilakukan penelitian lanjutan untuk mengetahui faktor-faktor yang memengaruhi beban pramurawat tersebut.

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Background: Being caregiver of geriatric patient lead the risk for developing of health and mental health conseguences and also caregiver burden. This study is aim to analysis asscociated dependecy and caregiver burden of geriatric patient.

Method: This is a cross-sectional study of 116 caregiver of geriatric out patient in Geriatric Holistic Clinic at Cipto Mangunkusumo Hospital. The study went on July-August 2013. All of the caregivers filled the questioner of The Zarith Burden Interview (ZBI) and questioner of Activity Daily Living (ADL) filled by reseacher.

Result: this study found that mean average of Activity Daily Living (ADL) of geriatric patient is 13 (SD 16, min-maks 0-20). Average mean of caregiver burden is 29,2 (10-61). There's a kcorelation beetween score of ADL and caregiver burden score (p < 0,001). Corrrelation value -0,381 showed the menunjukkan bahwa arah korelasi negatif dengan kekuatan korelasi lemah.

Conclusion: There is reciprocal significant association between degree of kemandirian and caregiver burden with weak correlation."

"Defisiensi vitamin D sering terjadi pada penyakit autoimun, termasuk pemfigus vulgaris (PV) dan systemic lupus erythematosus (SLE). Sementara itu, terapi nutrisi dan suplementasi vitamin D masih belum rutin dilakukan dalam tata laksana PV dan SLE. Serial kasus ini melaporkan terapi nutrisi dan suplementasi vitamin D pada empat kasus penyakit autoimun yang mengalami kekambuhan. Serial kasus terdiri atas dua pasien laki-laki PV dan dua pasien perempuan SLE dengan defisiensi vitamin D yang putus obat akibat pandemi corona virus disease 2019 (COVID-19). Keempat pasien mengalami malnutrisi berat secara klinis, karena penurunan asupan makanan dan berat badan dengan berbagai komplikasi obat imunosupresan jangka panjang, yaitu meningkatnya risiko infeksi, sepsis, sarkopenia, deposisi lemak, diabetes mellitus diinduksi steroid, dislipidemia, hipertensi, dan depresi. Asupan energi secara bertahap ditingkatkan secara enteral melalui nasogatric tube (NGT) dan/atau rute oral untuk memenuhi kebutuhan energi dan protein total. Kebutuhan energi total menggunakan Formula Harris-Benedict dengan faktor stres yang disesuaikan dengan profil klinis dan metabolik masing-masing pasien. Kebutuhan protein ditetapkan 1,5–2,0 g/kg BB/hari untuk pasien PV dan 0,8–1,2 g/kg BB/hari untuk pasien SLE dengan keterlibatan ginjal. Lemak dan karbohidrat (KH) disesuaikan dengan komposisi seimbang, yaitu 45–60% KH, 25 g serat, dan <5% added sugar serta 25–30% lemak dengan <7% asam lemak jenuh, ~20% asam lemak tak jenuh tunggal, dan ~ 10% asam lemak tak jenuh jamak. Dua pasien PV mengalami insufisiensi (16,4 ng/mL dan 22,1 ng/mL) dan dua pasien SLE mengalami defisiensi (6,6 ng/mL dan 9,1 ng/mL). Keempat pasien mendapatkan kolekalsiferol 6000 IU/hari selama 8 minggu berturut-turut. Setelah 1 bulan suplementasi vitamin D dan terapi nutrisi adekuat, serum vitamin D serta status nutrisi dan skor Karnofsky meningkat. Kualitas hidup yang dinilai dengan Dermatology Life Quality Index (DLQI) untuk pasien PV dan Lupus quality of life (LupusQoL) untuk pasien SLE juga meningkat. Serial kasus ini menyimpulkan bahwa tata laksana komprehensif yang menyertakan terapi nutrisi adekuat dan evaluasi serum vitamin D dapat meningkatkan kondisi klinis dan metabolik, status gizi, kapasitas fungsional, dan kualitas hidup pasien autoimun kambuh.

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Vitamin D deficiency is common in autoimmune disease, including pemphigus vulgaris (PV) and systemic lupus erythematosus (SLE). Meanwhile, nutrition therapy and vitamin D supplementation are still not routines in comprehensive management of PV and SLE. In this case series, we report nutrition therapy and vitamin D supplementation of four cases of relapse autoimmune disease. This series consist of two males of PV and two females of SLE with vitamin D deficiency that dropped out of treatment due to corona virus disease 2019 (COVID-19) pandemic. Patients became clinically severe malnutrition because of reduced food intake and body weight with various long-term immunosuppressant drug complications, ie increased risk of infections, sepsis, sarcopenia, fat deposition, steroid induced diabetes mellitus, dyslipidemia, hypertension, and depression. Energy intake was gradually increased enterally via nasogatric tube (NGT) and/or oral route to meet total energy and protein requirement. Total energy requirement was calculated by Harris-Benedict Formula with stress factor adjusted by clinical and metabolic profile of each patient. Protein requirement set by 1.5–2.0 g/kg BW/day for PV and 0,8–1,2 g/kg BW/day for SLE with renal involvement. Fat and carbohydrate (CHO) were tailored by balance composition, ie 45–60% CHO, 25 g fiber, and <5% added sugar and 25–30% fat with <7% saturated fatty acid, ~20% monounsaturated fatty acid, and ~10% polyunsaturated fatty acid. Two PV patients were insufficiency (16,4 ng/mL and 22,1 ng/mL) and two SLE patients were deficiency (6,6 ng/mL and 9,1 ng/mL). Cholecalciferol 6000 IU/day was prescribed for 8 weeks. After 1 month vitamin D supplementation and an adequate nutrition therapy, serum vitamin D was increased as well as nutritional state and Karnofsky’s score. Dermatology Life Quality Index (DLQI) for PV and LupusQoL for SLE were also enhanced. Finally, comprehensive management along with an adequate nutrition therapy and vitamin D evaluation improved clinical and metabolic condition, nutritional status, functional capacity, and quality of life of relapse autoimmune patient."