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Approach to the patient with acute severe autoimmune hepatitis.

ABSTRACT: Autoimmune hepatitis is associated with varied clinical presentations and natural history, as well as somewhat unpredictable treatment responses. Understanding how to stratify patients who require further escalation of therapy will help clinicians manage these patients. The presentation of acute severe autoimmune hepatitis (AS-AIH) is relatively uncommon, although its prevalence is potentially greater than currently perceived. Previous studies consist of small retrospective single-centre series and are not directly comparable due to the diversity of presentations, disease definitions and non-standardised treatment regimens. We define AS-AIH as those who present acutely with AIH and are icteric with an international normalised ratio ≥1.5 and no evidence of hepatic encephalopathy. Those with hepatic encephalopathy should be defined as having AS-AIH with acute liver failure. In this review, we provide a structured practical approach for diagnosing and managing this unique group of patients.

SUBMITTER: Rahim MN

PROVIDER: S-EPMC7509236 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Approach to the patient with acute severe autoimmune hepatitis.

Rahim Mussarat N MN Miquel Rosa R Heneghan Michael A MA

JHEP reports : innovation in hepatology 20200721 6

Autoimmune hepatitis is associated with varied clinical presentations and natural history, as well as somewhat unpredictable treatment responses. Understanding how to stratify patients who require further escalation of therapy will help clinicians manage these patients. The presentation of acute severe autoimmune hepatitis (AS-AIH) is relatively uncommon, although its prevalence is potentially greater than currently perceived. Previous studies consist of small retrospective single-centre series ...[more]

PMID: 32995712

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Project description:We present a 55-year-old male patient - a smoker, admitted to a Medical Institute of MVR (Ministry of the interior, Sofia, Bulgaria), on occasion of pain and swellings, located in the area of both axillae, accompanied by purulent discharge, with bloody admixtures. Bilateral localised cystic rose above the skin surface, hyperpigmented nodules interconnected with multiple fistulas, was observed within the dermatological examination, resulting in a limitation of the possibility of movement of the hands in all directions. A subjective complaint of pain was obtained on palpation. Solid bilateral axillar cicatrices - formation was also established, which additional impeded the movements of the upper limbs. The disease was generalised affecting additional inguinal, femoral and perineal areas, while at this stage the patient refused categorically eventual photo documentation of them. The diagnosis of acne inversa was made based on the available clinical and para-clinical data, as dual antibiotic therapy with Clindamycin 300 mg, two times per day was initiated for two months, in combination with rifampicin 300 mg, two times per day also for two months. This led to a significant improvement in the clinic symptoms and the patient was hospitalised for radical surgery. A surgical management of the clinical findings was planned by an interdisciplinary team including surgeons and dermatologists. The procedure was performed under general anaesthesia. After a thorough cleaning of the operative field, a radical excision of the lesion in the left axillary and para axillar region was performed, comprising the skin and subcutaneous tissue forward the fascia pectoralis. Tissue was dissected in depth in the form of number 4, thereby creating the conditions for adaptation of the initially encountered communicating with each other skin defects. Two tubular drains were placed, followed by gradual suturing of skin and subcutaneous tissue with final applying of a sterile dressing. Effective medical treatment of patients (as in our case) with severe AI is limited. Adalimumab is the first biological approved for moderate to severe AI but does not result in stable CR (cure rate). Therefore its use in a neoadjuvant setting is under investigation. Wide local excision significantly reduces pain and improves the quality of life of AI patients. While local recurrences rate is low, the satisfaction with the cosmetic results is high. The recurrence rate is dependent on the region affected and the type of surgery. While in the axillary region primary closure may be used to reduce the time to healing, anogenital AI has the lowest recurrence rate of healing by secondary intention.

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Approach to the patient with acute severe autoimmune hepatitis.

Publications

Approach to the patient with acute severe autoimmune hepatitis.

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