Clinical characteristics and treatment outcomes of acute severe autoimmune hepatitis

Abstract Background and aim Acute severe autoimmune hepatitis (AS-AIH) is a rare cause of acute liver failure (ALF), which is often neglected and delayed in treatment. The purpose of this study was to analyze the clinical characteristics and therapeutic effects of AS-AIH. Methods Retrospective analysis was performed. AIH was diagnosed according to the International Autoimmune Hepatitis Group (IAIHG) criteria revised in 1999. AS-AIH was defined as an acute presentation (onset of symptoms to presentation of ≤ 26 weeks) and INR of ≥ 1.5, and no histologic evidence of cirrhosis. Results Twelve patients were diagnosed as AS-AIH. At baseline, median immunoglobulin G was 28.35 g/L (range, 11.4–49.2). Ten (83.3%) patients were antinuclear antibodies and/or anti-smooth muscle antibodies positive. The prominent histologic characteristics were lobular necrosis/inflammation (91.7%) and plasma cell infiltration (100%). All patients received corticosteroid therapy. Death occurred in 2 (16.7%) patients within 30 days resulted from ALF. The average interval between the onset of symptoms and initiation of corticosteroid therapy in deceased patients was 65 days, compared with 19 days for survivors. Conclusions AS-AIH is an uncommon disease with poor outcomes. Patients with acute severe hepatitis of unknown cause should be minded the possibility of AS-AIH and corticosteroids should be considered as soon as possible.

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Clinical characteristics and corticosteroid therapy in patients with autoimmune-hepatitis-induced liver failure

World Journal of Gastroenterology ◽

10.3748/wjg.v20.i23.7473 ◽

2014 ◽

Vol 20 (23) ◽

pp. 7473 ◽

Cited By ~ 4

Author(s):

Bing Zhu

Keyword(s):

Liver Failure ◽

Autoimmune Hepatitis ◽

Corticosteroid Therapy ◽

Clinical Characteristics

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Autoantibodies in Autoimmune Hepatitis

Digestive Diseases ◽

10.1159/000440748 ◽

2015 ◽

Vol 33 (Suppl. 2) ◽

pp. 65-69 ◽

Cited By ~ 8

Author(s):

Luigi Muratori ◽

Gaia Deleonardi ◽

Claudine Lalanne ◽

Erica Barbato ◽

Alessandra Tovoli ◽

...

Keyword(s):

Smooth Muscle ◽

Autoimmune Hepatitis ◽

Validation Studies ◽

Antinuclear Antibodies ◽

Recombinant Antigens ◽

Liver Cytosol ◽

Diagnostic Score ◽

Clinical Correlations ◽

Smooth Muscle Antibodies

Background: The detection of diagnostic autoantibodies such as antinuclear antibodies (ANA), anti-smooth muscle antibodies (SMA), anti-liver/kidney microsomal type 1 (anti-LKM1), anti-liver cytosol type 1 (anti-LC1) and anti-soluble liver antigen (anti-SLA) is historically associated with the diagnosis of autoimmune hepatitis. Key Messages: When autoimmune hepatitis is suspected, the detection of one or any combination of diagnostic autoantibodies, by indirect immunofluorescence or immuno-enzymatic techniques with recombinant antigens, is a pivotal step to reach a diagnostic score of probable or definite autoimmune hepatitis. Conclusions: Diagnostic autoantibodies (ANA, SMA, anti-LKM1, anti-LC1, anti-SLA) are a cornerstone in the diagnosis of autoimmune hepatitis. Other ancillary autoantibodies, associated with peculiar clinical correlations, appear to be assay-dependent and institution-specific, and validation studies are needed.

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Meta-Analysis: Diagnostic Accuracy of Antinuclear Antibodies, Smooth Muscle Antibodies and Antibodies to a Soluble Liver Antigen/Liver Pancreas in Autoimmune Hepatitis

PLoS ONE ◽

10.1371/journal.pone.0092267 ◽

2014 ◽

Vol 9 (3) ◽

pp. e92267 ◽

Cited By ~ 17

Author(s):

Wen-Chao Zhang ◽

Feng-Rong Zhao ◽

Juan Chen ◽

Wei-Xian Chen

Keyword(s):

Smooth Muscle ◽

Diagnostic Accuracy ◽

Autoimmune Hepatitis ◽

Antinuclear Antibodies ◽

Meta Analysis ◽

Smooth Muscle Antibodies

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Statin-induced necrotising autoimmune myopathy and autoimmune hepatitis presenting with dysphagia

BMJ Case Reports ◽

10.1136/bcr-2019-232391 ◽

2020 ◽

Vol 13 (2) ◽

pp. e232391 ◽

Cited By ~ 1

Author(s):

Osama Qasim Agha ◽

Sukhdeep Kaur ◽

Nirmal Vijayavel

Keyword(s):

Creatine Kinase ◽

Autoimmune Hepatitis ◽

Complete Resolution ◽

Oropharyngeal Dysphagia ◽

Liver Function Tests ◽

Unknown Aetiology ◽

Proximal Muscle Weakness ◽

Proximal Muscle ◽

Autoimmune Myopathy ◽

Smooth Muscle Antibodies

Statin-induced necrotising autoimmune myopathy (SINAM) is a rare disease characterised by proximal muscle weakness and elevated creatine kinase levels that is usually in the thousands. Anti-3-hydroxy-3-methyl glutaryl co-enzyme A reductase (HMGCR) antibodies are associated with SINAM. Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is usually of unknown aetiology but can also be associated with concurrent extrahepatic autoimmune disorders. We are reporting a case of biopsy proven AIH associated with SINAM in a patient presenting with oropharyngeal dysphagia. The patient had elevated anti-HMGCR antibodies and anti-smooth muscle antibodies. SINAM and AIH were confirmed by muscle biopsy and liver biopsy, respectively. The patient had complete resolution of his symptoms and complete normalisation of his liver function tests after 6 months of the treatment.

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Capsular warning syndrome: clinical analysis and treatment

BMC Neurology ◽

10.1186/s12883-019-1522-0 ◽

2019 ◽

Vol 19 (1) ◽

Author(s):

Lanying He ◽

Ronghua Xu ◽

Jian Wang ◽

Lili Zhang ◽

Lijuan Zhang ◽

...

Keyword(s):

Clinical Characteristics ◽

Intravenous Thrombolysis ◽

Internal Capsule ◽

Neurological Impairment ◽

Clinical Analysis ◽

Good Prognosis ◽

Clinical Syndrome ◽

Therapeutic Effects ◽

Acute Infarction ◽

Capsular Warning Syndrome

Abstract Background Capsular warning syndrome (CWS) is a rare clinical syndrome, which is defined as a recurrent transient lacunar syndrome. The mechanism and clinical management of CWS remain unclear. The aim of the study was to discuss the clinical characteristics of CWS and evaluate the different outcome between rt-PA and no rt-PA therapy. Methods The present multicenter retrospective study involved three medical centers, and the clinical data were collected from patients with CWS between January 2013 and December 2018. The clinical characteristics of CWS were analyzed. Patients were divided into two groups: rt-PA and no rt-PA groups. The therapeutic effects and prognosis of these two groups were analyzed. A good prognosis was defined as 3-month modified Rankin Scale (mRS) ≤ 2. Results Our study included 72 patients, 27 patients were assigned to rt-PA group, 45 in no rt-PA group. Hypertension and dyslipidemia were the most common risk factors. The mean number of episodes before irreversible neurological impairment or the symptoms completely disappeared was five times (range: 3–11 times). A total of 58 (80.55%) patients had acute infarction lesions on the diffusion weighted imaging (DWI). The most common infarct location was the internal capsule (41,70.69%), followed by the thalamus and pons. The difference in therapeutic effects between the rt-PA, single and double antiplatelet groups was not statistically significant (P > 0.05). A good prognosis was observed in 61 (84.72%) patients after 3 months, in which 23 (23/27, 85.19%) patients were from the rt-PA group and 38 (38/45,84.44%) patients were from the no rt-PA group (P > 0.05). After 3 months of follow-up, two patients had recurrent ischemic stroke. Conclusion The most effective treatment of CWS remains unclear. Intravenous thrombolysis is safe for CWS patients. Regardless of the high frequency of infarction in CWS patients, more than 80% patients had a favorable functional prognosis.

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Keratitis occurring in patients treated with miltefosine for post-kala-azar dermal leishmaniasis

British Journal of Ophthalmology ◽

10.1136/bjophthalmol-2020-317325 ◽

2020 ◽

pp. bjophthalmol-2020-317325

Author(s):

Rakhi Kusumesh ◽

Anita Ambasta ◽

Lalan Kumar Arya ◽

Nilesh Mohan ◽

Bibhuti Prassan Sinha ◽

...

Keyword(s):

Corticosteroid Therapy ◽

Causality Assessment ◽

Hand Movement ◽

Ocular Symptoms ◽

Kala Azar ◽

Microbiological Characteristics ◽

Improved Outcomes ◽

Corrected Distance Visual Acuity ◽

Poor Outcomes ◽

Ulcerative Keratitis

AimTo describe the characteristic clinical features and management of keratitis in the patients receiving miltefosine for post-kala-azar dermal leishmaniasis (PKDL).MethodsThe medical records of five patients with PKDL who presented with keratitis were reviewed retrospectively from April 2018 to December 2019. The evaluation included a thorough medical history including details on drugs used, particularly miltefosine. The drug causality assessment was also performed. The clinical and microbiological characteristics of keratitis were noted.ResultsThe ocular symptoms included pain, redness, watering, photophobia and diminution of vision. Slit-lamp biomicroscopy revealed peripheral, paralimbal, ring-shaped, full-thickness stromal infiltration resulting in ulcerative keratitis in all cases. Two patients had unilateral keratitis, while three had bilateral keratitis. All five patients received miltefosine for an average period of 48 days before the onset of keratitis. The corrected distance visual acuity at presentation ranged from hand movement to 20/125. The causality assessment revealed a ‘probable’ association between the adverse drug reaction and miltefosine in all patients. Discontinuation of miltefosine and initiation of corticosteroid therapy resulted in resolution of keratitis in all cases. The unilateral keratitis treated with topical corticosteroids had improved outcomes, but poor outcomes were found in the bilateral keratitis.ConclusionThese observations indicate that prolonged use of miltefosine might cause keratitis that resembles infectious keratitis. Early diagnosis with discontinuation of the drug and initiation of corticosteroid therapy are the key to successful management.

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