Polyarteritis nodosa presenting as cholecystitis-a case report.

Medium and small arteries are mainly affected by polyarteritis nodosa. Lungs are spared but any other organ can be involved. Gallbladder can be part of this systemic disease. Isolated gallbladder disease is not common. The presentation of the systemic polyarteritis nodosa as acute cholecystitis is described in this case report. Management of the disease depends on the involved organs and usually consists of systemic steroids. The diagnosis of polyarteritis nodosa should be considered in patients with previous systemic symptoms who develop picture of acute cholecystitis.

Tumefactive Demyelination: A Common Radiological Masquerader of Malignancy and the Expeditious Diagnostic Applicability of Crush Smear Cytology.

Tumefactive demyelinations (TDs) are demyelinating central nervous system lesions that masquerade as neoplastic lesions on radiological images. Brain biopsy is often required for confirmatory diagnosis. Since crush cytology has become a routine practice, a thorough knowledge of the cytomorphologic features of TD is required to prevent misdiagnosis. In this report, we describe the cytomorphological and histomorphological features of a case of TD.

Syringomatous Adenoma of the Nipple in a Male Breast: A Case Report With a Brief Review of Literature and Histomorphological Approach to Diagnosis.

Syringomatous adenoma of the nipple (SAN) is a benign and locally infiltrative lesion possibly arising from the sweat gland ducts in the nipple-areolar region. This rare lesion has been reported in the female breast; however, reports on the male breast are extremely rare. Although benign, SAN has a high risk of recurrence. The clinical presentation and histomorphological features often mimic a malignancy. Hence, an awareness of this lesion is required to make a correct diagnosis. In this report, we describe the histomorphological features of SAN in a male breast.

Repurposing Pirfenidone for Nonalcoholic Steatohepatitis-related Cirrhosis: A Case Series.

We repurposed the antifibrotic drug pirfenidone-which is approved for treatment of idiopathic lung fibrosis-in a series of patients with nonalcoholic steatohepatitis-related cirrhosis. Our report demonstrates the observed improvements in necroinflammation and regression of cirrhosis with pirfenidone use for 12-weeks, associated with classical hepatic repair complex features on follow-up liver biopsies. This novel work could help stimulate further randomized trials of pirfenidone in patients with nonalcoholic steatohepatitis-related liver fibrosis or cirrhosis, for whom no recommended drug treatments exists currently.

Granulocyte Colony-Stimulating Factor Use in Decompensated Cirrhosis: Lack of Survival Benefit.

BACKGROUND: Granulocyte colony-stimulating factor (GCSF) has been utilized in decompensated cirrhosis (DC) for improving transplant-free survival (TFS). Data from multiple centers are conflicting with regard to patient outcomes. In this retrospective study, we present our 'real-world experience' of GCSF use in a large group of DC. METHODS: From September 2016 to September 2018, 1231 patients with cirrhosis were screened, of which 754 were found to have decompensation(s). Seventy-three patients with active ascites, jaundice, or both completed GCSF treatment (10 mcg/kg per day for 5 days, followed by 5 mcg/kg/day once every third day for total 12 doses). Per-protocol analysis (n = 56) was performed to study clinical events, liver disease severity, and outcomes at 3, 6, and 12 months after treatment. Modified intention-to-treat (mITT, n = 100) analysis was performed to study overall survival at 180 days. Outcomes were compared with a matched historical control (HC) group (n = 24). RESULTS: Nine (16%, n = 56), 24 (43%, n = 56), and 36 (75%, n = 48) patients died at 3, 6, and 12-month follow-up after GCSF. The commonest cause of death was sepsis (53%) followed by progressive liver failure (33%). Nine percent of patients developed hepatocellular carcinoma on follow-up at the end of 1 year. Acute variceal bleeds, overt hepatic encephalopathy, intensive unit admissions, and liver disease severity scores were higher after treatment at the end of 1 year. The Child-Pugh score >11 and model for end-stage liver disease-sodium score >25 and > 20 predicted worse outcomes at all time points and at 6 and 12 months after GCSF, respectively. Compared to a matched HC group, patients receiving GCSF had higher mortality (75% vs 46%, P = 0.04) at one year. mITT analysis revealed poor overall survival at 6 months compared to HCs (48% vs 75%, P = 0.04). CONCLUSION: Survival in DC was shorter than what was expected in the natural history of the disease after GCSF use.

A Single-Center Experience on Outcomes of Complementary and Alternative Medicine Use Among Patients With Cirrhosis.

Drug-induced liver injury (DILI) due to complementary and alternative medicine (CAM) use is on the rise throughout the world by patients looking for "safer" alternatives. However, data on acute-on-chronic liver failure (ACLF) due to CAM are lacking. In a large cohort of patients with cirrhosis, we retrospectively studied CAM-related health-seeking behavior and attempted to identify those who developed possible CAM-DILI-related ACLF. In this study, we examine the clinical, biochemical, and liver histopathologic characteristics of possible CAM-DILI-related ACLF, describe implicated CAM agents, and discuss predictors of patient outcomes. Out of 1,666 patients with cirrhosis, 68% used CAM at some point. A total of 35.7% (n = 30/84) patients presented with CAM-related DILI leading to ACLF in the whole CAM-DILI-related decompensation cohort. The most common CAM was unlabeled polyherbal Ayurvedic formulations. Of possible patients with ACLF, 63% self-medicated with CAM based on social media sharing. Mean age ± SD was 51.9 ± 9.9 years, 83% were male patients, median follow-up duration was 173 (range, 14-584) days, median Child-Turcotte-Pugh score was 13 (range, 10-14), Model for End-Stage Liver Disease-sodium score was 30.1 ± 4.8, median chronic liver failure-organ failure (CLIF-C-OF) score was 11 (range, 8-14), and median CLIF-C-ACLF score was 98 (range, 87-127). Portal-based neutrophilic predominant mixed inflammation, hepatocyte ballooning, autoimmune-like features, and severe cholestasis were seen on liver biopsy. Overall, 53% of patients died (median survival 194 days). Baseline overt hepatic encephalopathy and CLIF-C-OF score, total bilirubin, hyponatremia and leukocytosis, and grade of ACLF predicted 1-, 3-, 6- and 12-month mortality, respectively. Conclusion: Possible CAM-DILI-related ACLF has a high mortality. Strict monitoring and identification of CAM use among people with cirrhosis and an integrative public health educational practice can help ameliorate this modifiable risk factor that potentiates heavy liver disease burden and resource use.

Ayurveda Herbal Medicine-induced Liver Cirrhosis.

We present the rare case of a young male with sinusoidal obstruction syndrome due to Ayurvedic herbal medicine which he took for management of bilateral leg swelling associated with protein-losing enteropathy due to intestinal lymphangiectasia. The patient developed progressive sinusoidal fibrosis leading to cirrhosis on long term follow-up. In a diagnosis that took three years to conclude, we showcase serial liver biopsies that reveal the rare disease progression. Complementary and alternative medicine use among apparently healthy population is a potentially modifiable risk factor for liver diseases, in the presence of adequate public health education from concerned authorities.

Homeopathy-medicine induced severe alcoholic hepatitis.

We present a teetotaler with compensated non-alcoholic fatty-liver-disease related cirrhosis who presented with acute worsening of his chronic liver disease. The acute event was not discernible even after extensive work up and finally a transjugular liver biopsy revealed features suggestive of severe alcoholic hepatitis. The patient and the family denied occult alcohol use when questioned over multiple times and finally, the culprit 'alcohol' was found to be the homoeopathy medicines that the patient was consuming over a month for treatment of Gilbert's syndrome. We retrieved and tested the homoeopathy drug for alcohol content and found an alarming 18% ethanol in the same, confirming our diagnosis.

Ayurveda metallic-mineral 'Bhasma'-associated severe liver injury.

Ayurveda Bhasma is a metallic-mineral preparation homogenised with herbal juices or decoctions and modified with heat treatment to apparently detoxify the heavy metals. It is widely recommended for the treatment of many disease conditions by practitioners of complementary and alternative medicine in the absence of good quality clinical trial evidence on its safety and efficacy. Heavy metal-induced liver injury is widely reported in the literature, and heavy metal adulteration of non-Bhasma-related Ayurveda and herbal products has been well described. We report a patient who developed severe liver injury requiring listing for liver transplantation for improved survival, after consumption of Bhasma for dyspepsia. This case describes the first documented case and toxicology analysis of Ayurveda Bhasma associated with severe drug-induced liver injury. Physicians must be alert regarding patient's use of supposedly safe Ayurveda Bhasma that may promote acute severe liver injury in the absence of other known aetiologies.

Clinical outcomes, histopathological patterns, and chemical analysis of Ayurveda and herbal medicine associated with severe liver injury-A single-center experience from southern India.

INTRODUCTION: Ayurvedic and herbal medicines (AHM) are known to cause varying degrees of drug-induced liver injury (DILI). Clinical, biochemical, histological spectrum and outcomes of AHM linked to severe DILI are not well studied. METHODS: Out of 1440 liver disease patients, 94 were found to have a severe liver injury and associated AHM intake. Thirty-three patients were suspected to have AHM-DILI on Roussel Uclaf Causality Assessment Scoring Method. Forty-seven and 30 of retrieved AHM samples were analyzed for heavy metals and hepatotoxic volatile organic compounds (hVOCs), respectively. Eleven patients ingested AHM from unregistered traditional healers (UTH). Clinicopathological outcomes were analyzed in 27 patients (who underwent liver biopsy) and outcomes with respect to chemical analyses were studied in 33 patients. RESULTS: Males predominated (70.4%) with mean age 46.9±15.8 years. Mean follow up was 119.2±81.4 days. The median duration of drug intake was 28 days (10 - 84). Five patients died (18.5%). Hepatic encephalopathy, hypoalbuminemia, and hepatic necrosis were significantly associated with mortality (p < 0.005). Arsenic and mercury ingestion was significantly associated with death (p < 0.005). hVOCs were detected in more than 70% of samples. AHM intake from UTH was associated with higher mortality. CONCLUSION: Adequate regulation and scrutiny regarding AHM use among the general population is an unmet need. Early liver biopsy after clinical identification of at-risk patients can expedite definitive treatment with a liver transplant.

Strange case of dimorphic skin rash in a patient with cirrhosis: atypical herpes simplex and sweet's syndrome.

A middle-aged man with decompensated cirrhosis and a dimorphic multisite skin rash is diagnosed with rare atypical herpes simplex infection, manifesting Sweet's syndrome (SS) in the absence of other described associations. SS, an acute febrile neutrophilic dermatosis, has three common forms-classical or idiopathic, malignancy associated and drug induced. Systemic autoimmune, connective tissue diseases and infections are also strong associations. The latter is commonly described in Gram-positive bacteria, salmonellosis and Yersinia Herpes infections are a rare cause of SS, reported only thrice in literature, one with concomitant lupus, the second with associated mycobacterial infection and third, in metastatic breast disease in contrast to our patient. Atypical rash, especially if dimorphic, warrants histopathological evaluation to confirm underlying disease.