Background: There is a dearth of data on Filipino patients with autoimmune hepatitis (AIH). We aimed to describe the demographic and clinical profiles of patients with AIH and to characterize clinical outcomes and treatment responses. Methods: A retrospective cohort study involving patients from two tertiary centers diagnosed with AIH from January 1, 2007, to December 31, 2019, was included. Disease remission was defined as the normalization of ALT levels, while failure was defined as an increase in ALT levels over baseline or clinical deterioration. Results: A total of 48 patients were identified between 2007 to 2019. The median age at presentation was 51 (27-79 yrs.). Liver cirrhosis was already present in 37.5% (27.1% decompensated) on diagnosis. Aside from a higher histologic activity index in females (p=0.047), there were no gender-specific differences. Disease remission was achieved in 41.9% of patients at 6 months, while only 9.3% failed. At the final disposition, remission rates had slightly increased to 58%, but failure rates had risen to 12%. Treatment responses at both 6 and 12 months and MELD and Child-Pugh class influenced treatment responses at final disposition. Median overall survival was 102 weeks and was influenced by the presence of liver dysfunction and 12 months and final treatment responses. Conclusion Autoimmune hepatitis remains an important cause of morbidity and mortality. The results of the study highlight the need for immunosuppressive therapy to induce early remission for a higher likelihood of subsequent biochemical remission to reduce the risk of liver-related mortality.
Autoimmune Hepatitis ; Liver Cirrhosis ; Steroids ; Azathioprine

Introduction: Epidermolysis Bullosa Acquisita (EBA) is a rare autoimmune blistering disease which presents in the skin and mucous membranes. The decrease in anchoring fibrils in the basement membrane zone causes separation of the epidermis from the dermis, resulting in its blistering presentation. The treatment plan will depend on the severity of the disease. The first-line treatment for mild EBA includes topical corticosteroids and immunomodulators such as dapsone and colchicine; while severe cases of EBA may be given intravenous immunoglobulins, systemic steroids, and immunosuppressants such as azathioprine and cyclophosphamide. Case Report: This is a case of a 50-year-old Filipino male who presented with a 2-year history of vesicles and tense bullae which evolved into papules, plaques and erosions with scarring and milia formation on the scalp and trauma-prone areas of the trunk and extremities. Clinical examination revealed multiple, well-defined, irregularly shaped erythematous papules and plaques with crusts, scales, erosions, pearl-like milia and scarring on the chest, back, upper, and lower extremities. The oral mucosa was moist with some ulcers on the tongue. Histopathologic examination using Hematoxylin and Eosin (H&E) stain revealed the absence of the epidermis with retention of dermal papillae suggestive of subepidermal clefting. Further examination with direct immunofluorescence (DIF) revealed monoclonal immunoglobulin (IgG) deposits demonstrating an intense linear fluorescent band at the dermoepidermal junction, consistent with Epidermolysis Bullosa Acquisita. Overall, the combined administration of prednisone, azathioprine, and colchicine resulted only in transient and incomplete resolution of lesions in this case of EBA. Conclusion The management of EBA is mostly supportive with the goal of minimizing complications. Combination treatments using steroids, colchicine, and azathioprine have been reported with various results. Its management remains challenging as most cases are refractory to treatment.
Epidermolysis Bullosa Acquisita ; bullous disease ; azathioprine ; colchicine ; prednisone

This study aimed to evaluate the efficacy and safety of mycophenolate mofetil (MMF) or tacrolimus (TAC) compared with azathioprine (AZA) as maintenance therapy for active lupus nephritis (ALN). Patients with ALN who responded to 24 weeks of induction treatment were enrolled. Patients who received MMF or TAC as induction therapy continued MMF or TAC treatment during the maintenance period, whereas those who received intravenous cyclophosphamide were subjected to AZA treatment. The primary endpoint was the incidence of renal relapse. Secondary endpoints included extrarenal flares and composite endpoints (deaths, end-stage renal disease, or doubling of serum creatinine levels). A total of 123 ALN patients (47 in the MMF group, 37 in the TAC group, and 39 in the AZA group) were enrolled. The median follow-up time was 60 months. Ten MMF-treated patients, ten TAC-treated patients, and eight AZA-treated patients experienced renal relapses (P = 0.844). The cumulative renal relapse rates in the MMF group (P = 0.934) and TAC group (P = 0.673) were similar to the renal relapse rate in the AZA group. No significant difference in the incidence of severe adverse event was observed among the groups. Long-term maintenance therapies with MMF or TAC might have similarly low rates of renal relapse and similar safety profiles compared with AZA.
Humans ; Mycophenolic Acid/adverse effects* ; Azathioprine/adverse effects* ; Tacrolimus/therapeutic use* ; Lupus Nephritis/complications* ; Immunosuppressive Agents ; Treatment Outcome ; Recurrence

Introduction: Pemphigus vulgaris is a life-threatening, autoimmune bullous disease caused by desmogleins (Dsg) 1 and 3 au- toantibodies. It is a rare disease with an incidence rate of 0.5 to 3.2 per 100,000 per year. It typically presents as painful, flaccid blisters and erosions on both the skin and mucous membranes. Case report: We present a 43-year-old male with painless penile erosions of 1-month duration. He was evaluated for sexually transmitted infections, but laboratory tests yielded negative results. Subsequently, vesicles and bullae on the back and hyper- keratotic lesions on the malar area appeared, leading to the differential diagnoses of bullous diseases. Skin biopsy was done revealing intraepidermal suprabasal blisters with acantholytic cells. Direct Immunofluorescence demonstrated positive inter- cellular deposits of IgG and C3. ELISA Dsg 1 and Dsg 3 were positive (ratio of 1.857 and 4.580, respectively). A final diagnosis of pem- phigus vulgaris (PV) was made. The patient has remained in remission after a 3-month course of prednisone and azathioprine. Conclusion This is a unique case of PV presenting with an unusual manifestation of painless penile erosions. There have been limited reports of PV with penile skin involvement and all cases presented with painful lesions. Because painless penile lesions as presenting feature is rare, the diagnosis may be easily missed. This case demonstrates that thorough dermatologic examination and early diagnosis despite atypical findings are crucial to provide timely and appropriate treatment as this determines the clinical outcome of the disease.
Pemphigus ; Desmogleins ; Azathioprine

OBJECTIVE: To investigate the association between single nucleotide polymorphism of NUDT15 gene (SNP rs116855232) and hepatotoxicity in children with acute lymphocytic leukemia (ALL). METHODS: A total of 135 children with ALL in Shandong Province were recruited in this study, and patients were divided into two groups based on the presence of liver injury. Genotypes of each patient were detected using PCR and Sanger sequencing. Clinical data and the average dose of 6-mercaptopurine (6-MP) were collected and analyzed by SPSS 19.0 software. RESULTS: Respectively, 99 patients were found with CC genotype, 32 patients with CT genotype and 4 patients with TT genotype. Compared with ALL patients without hepatotoxicity, there was a difference in genotypes between the two groups in the initial stage of chemotherapy for leukemia (Chi CONCLUSION The polymorphism of rs116855232 in NUDT15 gene was associated with hepatotoxicity induced by 6-mercaptopurine in children with ALL, and ALL patients with TT genotype should take a lower dose of 6-MP to avoided hepatotoxicity.
Antimetabolites, Antineoplastic/therapeutic use* ; Chemical and Drug Induced Liver Injury/genetics* ; Child ; Genotype ; Humans ; Mercaptopurine/adverse effects* ; Polymorphism, Single Nucleotide ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Pyrophosphatases/genetics*

azathioprine to achieve remission. Liver biopsy was performed at time of diagnosis for all patients and only 18 of them underwent a second biopsy after treatment.RESULTS: No statistically significant differences in the frequency of the genotypes GG and GC or in allele distribution were found in both patient and control groups (p=0.590, 0.640 respectively). Initial alanine aminotransferase (ALT) levels at the time of presentation was significantly higher in the GC group than GG group (p=0.020). GC genotype significantly correlated with disease relapse (r=0.41, p=0.007). Regression of necroinflammation and the fibrosis score in the second liver biopsy was statistically significant in the GG group (p < 0.0001, p=0.010 respectively).CONCLUSION: MIF -173GC polymorphism is associated with clinically significant markers of pediatric AIH, including increased initial serum ALT levels, may help predict necroinflammatory/fibrosis regression effectively, following immunosuppressive treatment.]]>
Alanine Transaminase ; Alleles ; Azathioprine ; Biopsy ; Child ; Chronic Disease ; Diagnosis ; Fibrosis ; Genotype ; Hepatitis, Autoimmune ; Humans ; Liver ; Macrophages ; Recurrence ; T-Lymphocytes

Immunoglobulin (Ig) G4-related disease (IgG4-RD) is newly recognized immune-mediated and fibroinflammatory conditions with various organ involvements. Any organs can be involved, but the pancreas, salivary gland, lymph nodes, and orbit are known to be commonly involved organs. A 54-year-old man presented with complaint of psoriasis like skin rash developed 4 years prior to admission. Although he had been treated for skin rash, the extent of skin lesions increased as well as hypereosinophilia, and multiple lymphadenopathies were newly developed. The patient was diagnosed with IgG4-RD by serum IgG4 levels and histologic examination of the inguinal lymph node. One month after treatment with steroid and azathioprine, his skin rash and lymphadenopathies resolved with improvement and eosinophil count was within the normal range. We herein report a case of a IgG4-RD patient associated with psoriasis-like skin rash and hypereosinophilic syndrome.
Azathioprine ; Eosinophils ; Exanthema ; Humans ; Hypereosinophilic Syndrome ; Immunoglobulin G ; Immunoglobulins ; Lymph Nodes ; Middle Aged ; Orbit ; Pancreas ; Psoriasis ; Reference Values ; Salivary Glands ; Skin

As an important drug during maintenance treatment of acute lymphoblastic leukemia (ALL), 6-mercaptopurine (6-MP) has several side effects, including hepatotoxicity and bone marrow suppression. Since its tolerability varies from person to person, 6-MP treatment should be individualized. The deficiency of thiopurine methyltransferase (TPMT) enzyme activity is associated with 6-MP intolerance. There is a lower frequency of mutation in TPMT alleles among Asian patients. Recent studies have shown that in ALL patients with NUDT15 gene mutation, the maximum tolerated dose of 6-MP is lower than the conventional dose. The article reviews the significance of NUDT15 gene in individualized treatment with 6-MP in children with ALL.
Antimetabolites, Antineoplastic ; Child ; Humans ; Mercaptopurine ; Methyltransferases ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Pyrophosphatases ; genetics

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Azathioprine ; Bronchiectasis ; Cough ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Dyspnea ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Immunosuppressive Agents ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Pathology ; Prognosis ; Survival Rate ; Traction

No abstract available.
Azathioprine ; Encephalomyelitis ; Myoclonus ; Steroids