Ursodeoxycholic acid for the treatment of hepatic sarcoid: A pre-post pilot study

Clinical and Experimental Hepatology · 2024-01-01

Aim of the study: Sarcoidosis is characterized by noncaseating granulomas that can affect multiple organs.Due to the lack of prospective studies regarding treatment of hepatic sarcoidosis with ursodeoxycholic acid (UDCA), we set out to evaluate its effects in a single-center, open-label, prospective, pre-post study.Material and methods: A total of 10 patients were screened from August 2018 to July 2020; seven met the criteria and were enrolled.The study was terminated prior to achieving target enrollment of 10 patients due to the difficulty in recruitment around the COVID-19 pandemic.Most patients were women (4/7; 57.1%) and African American (5/7; 71.4%).One patient dropped out during the first month of observation due to a new diagnosis of esophageal cancer.Six completed the 6-month observation and UDCA treatment periods.One patient stopped UDCA within the first month of active treatment due to the side effect of nausea.Results: There was a decrease in ALP and GGT after six months of UDCA treatment compared to six months of observation (ALP -257.6 to 202.2, p = 0.23; GGT -302.5 to 111.8, p = 0.059), but this did not reach statistical significance.There were also decreases in all key secondary endpoints (ALT -50.8 to 29.8, p = NS; AST -40.3 to 31.2, p = NS, VCTE kPa -8.3 to 6.3, p = NS).As with the primary endpoints, none of the key secondary endpoints reached statistical significance.Conclusions: There is significant potential for UDCA as first-line treatment of hepatic sarcoid.Multi-center, ideally prospective, studies of longer duration are needed.

An arm and a leg: A case of rheumatoid vasculitis and antiphospholipid antibody syndrome

SAGE Open Medical Case Reports · 2021-01-01 · 1 citations

Rheumatoid vasculitis is a rare extra-articular complication of rheumatoid arthritis. The most common manifestation is cutaneous; however, it can manifest in various organ systems and is associated with a high degree of morbidity and mortality. Diagnosis is challenging, and there are no validated diagnostic or classification criteria. Most cases should be confirmed with tissue biopsy when possible given the severity of disease and the extent of immunosuppression required to treat this condition. We report the case of a 54-year-old white woman with long-standing, uncontrolled, and seropositive rheumatoid arthritis with a history of elevated anticardiolipin IgG and IgM antibodies who presented with acute stenosis of her left femoral artery which ultimately required a left above-the-knee amputation. Histopathology revealed findings consistent with vasculitis and thrombosis, and subsequent imaging revealed multifocal arterial and venous thromboses. She was diagnosed with rheumatoid vasculitis and antiphospholipid antibody syndrome, and was treated with high-dose glucocorticoids, cyclophosphamide, and warfarin. Rheumatoid vasculitis is a rare but devastating complication of rheumatoid arthritis, and vigilance for this condition must be maintained, especially in patients with long-standing, seropositive disease.

THU0329 SAFETY, TARGET ENGAGEMENT, AND INITIAL EFFICACY OF AVID200, A FIRST-IN-CLASS POTENT AND ISOFORM-SELECTIVE INHIBITOR OF TGF-BETA 1 AND 3, IN PATIENTS WITH DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS (DCSSC): A PHASE 1 DOSE ESCALATION STUDY

Annals of the Rheumatic Diseases · 2020-06-01 · 17 citations

S2996 Granulomas Are the Clue

The American Journal of Gastroenterology · 2020-10-01

INTRODUCTION: Granulomatous diseases within the gastrointestinal (GI) tract make up a limited list of conditions. Identification of the exact pathology, however, isn’t always trivial particularly with atypical presentations, often requiring a multidisciplinary approach to arrive at a diagnosis. CASE DESCRIPTION/METHODS: Here, we describe a case of a 19-year-old man presented with recurrent hematemesis and 30 lbs weight loss over 4 months. He had a HR 114 and BP 84/61 and was thin-appearing without a tender abdomen. Labs were notable for Hgb 8g/dL (MCV 77, RDW 23.4) from baseline 12g/dL, WBC 7.7, Platelets 520, INR 1.5 and unremarkable liver enzymes. He was noted to have deficiencies in Folate, Vitamins D and C, and elevation in fecal calprotectin and IL-6. Computer Tomography of Chest, Abdomen and Pelvis demonstrated gastric wall thickening, small mediastinal and diffuse mesenteric lymphadenopathy. Upper endoscopy demonstrated thick prominent gastric folds with friable mucosa. Biopsies showed severe active chronic gastritis with ill-formed granulomas but no evidence of dysplasia or intestinal metaplasia. Extensive workup was consequently done including infectious studies, small bowel imaging, bone marrow biopsy, colonoscopy and mesenteric lymph node excisional biopsy revealing additional granulomas in duodenum and lymph nodes. The patient continued to develop hematemesis with poor tolerance of oral nutrition and vitamin deficiencies. Multidisciplinary discussion led to the final diagnosis of atypical sarcoidosis after excluding other competing diagnoses. The patient was started on daily budesonide with resolution of symptoms but persistent granulomas on follow-up gastric biopsies two months later. DISCUSSION: Diagnosis is complex in this patient. While Crohn’s disease can give a similar presentation, extensive lymphadenopathy in retroperitoneum would be associated with more extensive bowel inflammation which this patient lacked. Thus, sarcoid was favored after other granulomatous diseases were excluded. Though sarcoidosis rarely involves the GI tract in isolation, it led to linitis plastica-like appearance of gastric mucosa in this patient, causing gastric outlet obstruction which contributed to persistent vomiting, poor oral intake and weight loss, as well as gastric atrophy and erosions that resulted in hematemesis.Figure 1.: H&E slide of patient's gastric mucosa showing granuloma.Figure 2.: Gastric folds predominance on endoscopic retroflexion view.Figure 3.: Computer Tomography marked gastric wall thickening.

Tacrolimus induced pseudogout following allogeneic hematopoietic cell transplant

Journal of Oncology Pharmacy Practice · 2020-08-20 · 4 citations

Introduction Crystalline arthritis (CA), characterized by acute joint pain and erythema secondary to calcium pyrophosphate deposition (CPPD, or pseudogout) or monosodium urate crystals (gout), is a potentially underreported complication following allogeneic hematopoietic cell transplant (alloHCT). Graft-versus-host disease prophylaxis with calcineurin inhibitors (CNIs) causes hypomagnesemia and hyperuricemia, resulting in CA. CA related to tacrolimus has yet to be characterized following alloHCT. Case report We retrospectively reviewed records of 450 consecutive patients undergoing alloHCT and identified 15 (3.3% incidence) who developed CA on tacrolimus. Large joints were involved in 10 (66.7%) patients, all patients had recent hypomagnesemia, and no patient had hyperuricemia, suggesting CPPD was the most likely etiology. Management and outcome: Eleven (73.3%) patients received systemic corticosteroids; 6 as initial therapy and 5 added to or substituted for colchicine in the setting of slow or inadequate response. The median duration of corticosteroid therapy was 6 days, however 2 patients (13.3%) required prolonged maintenance due to recurrence. Eleven (73.3%) patients received colchicine; 9 as initial therapy and 2 added to or substituted for corticosteroids in the setting of slow or inadequate response. The median duration of colchicine therapy was 18 days. The median time to symptom resolution was 21 days. Discussion Patients on tacrolimus following alloHCT presenting with acute joint pain and erythema should be evaluated for CPPD. Hypomagnesemia secondary to CNIs is likely the precipitating factor for CPPD in this population. Patients can effectively be managed with systemic corticosteroids and/or colchicine, however prolonged duration of treatment and even maintenance may be necessary. Based on the Naranjo Algorithm, CPPD secondary to tacrolimus induced hypomagnesemia is a possible adverse drug event, with a score of 3–4.

The effect of vitamin D supplementation on glucose metabolism in type 2 diabetes mellitus: A systematic review and meta-analysis of intervention studies

Journal of Diabetes and its Complications · 2017-04-21 · 109 citations

Rates of Serious Infections in HIV‐Infected Patients Receiving Tumor Necrosis Factor Inhibitor Therapy for Concomitant Autoimmune Diseases

Arthritis Care & Research · 2016-06-22 · 20 citations

OBJECTIVE: To estimate the incidence of serious infections in patients with HIV infection and autoimmune disease who were treated with tumor necrosis factor (TNF) inhibitors, and to compare these rates by stratified viral load levels. METHODS: Using a unified search strategy, 4 centers identified HIV-infected patients exposed to TNF inhibitors. Patient characteristics and infection data were assessed via chart review in all patients who were ≥18 years old and who received TNF inhibitor therapy after HIV diagnosis, between January 1999 and March 2015. RESULTS: We studied 23 patients with 26 uses of TNF inhibitor therapy (86.7 person-years of followup). Two (8.7%) experienced at least 1 serious infection episode, for an overall incidence rate of 2.55 per 100 patient-years (95% confidence interval [95% CI] 0.28-9.23). The incidence rate per 100 patient-years was 3.28 (95% CI 0.04-18.26) among patients with a viral load >500 copies/ml at therapy initiation and 2.09 (0.03-11.65) among patients with a viral load ≤500 copies/ml. CONCLUSION: This study suggests that the rate of serious infections in patients with HIV infection under active care who have received treatment with TNF inhibitors may be comparable to the rates observed in registry databases.

Biomarkers in Scleroderma: Progressing from Association to Clinical Utility

Current Rheumatology Reports · 2016-03-01 · 16 citations

Oxalate nephropathy in systemic sclerosis: Case series and review of the literature

Seminars in Arthritis and Rheumatism · 2015-07-04 · 19 citations

A Wolf in Infection's Clothing: The Difficulties of Discerning Systemic Lupus Erythematosus from Its Infectious Sequelae

The American Journal of Medicine · 2015-02-03