Background Musculoskeletal (MSK) sarcoidosis presents with a variety of clinical phenotypes. Four subtypes of MSK sarcoidosis have been identified to date: Lofgren syndrome, chronic sarcoid arthritis, osseous sarcoidosis, sarcoid myopathy. Each subtype has been reported with varying incidence mainly due to lack of universal classification criteria. Methods We performed a retrospective chart review of patients with MSK sarcoidosis at a single academic center between January 2000 and December 2014. Descriptive statistics were used to describe the proportion of patients with sarcoidosis who had the 4 MSK syndromes of interest, demographic characteristics and therapeutic agents used. Results A cohort of 58 patients with MSK manifestations were identified among 1016 patients with sarcoidosis. Frequency of subtypes include: Lofgren syndrome 46.6%, osseous sarcoidosis 25.9%, chronic sarcoid arthritis 24.1% and sarcoid myopathy 6.9%. The cohort was predominantly female (43/58 patients, 74%) and Caucasian (48/58 patients, 82.8%). Mean age was 47.2 years. One patient had overlap of osseous sarcoidosis and chronic sarcoid arthritis, another patient initially had Lofgren syndrome and later developed chronic sarcoid arthritis. Sarcoid myopathy patients presented with myalgia more often than muscle weakness. Conclusion We identified a large cohort of MSK sarcoidosis and determined the prevalence of all 4 subtypes. In patients who do develop MSK manifestations of sarcoidosis, they are commonly a part of the initial presentation of sarcoidosis. There is an unmet need to establish standardized classification criteria for the 4 MSK sarcoidosis syndromes.
Background: Posttransplant tuberculosis (TB) is an uncommon complication following liver transplantation (LT). Given its high mortality, it is advocated to screen for latent TB with tuberculin skin test (TST), interferon gamma release assay and/or chest radiography before LT. Case Report: A 52-year-old Filipino gentleman was admitted with an 8-week history of abdominal pain, hematochezia, and weight loss. His pre-LT screening for latent TB with TST and chest radiography was negative. Colonoscopy revealed an ulcerated polypoid lesion in the terminal ileum. The cause of ulceration was histologically indeterminate. Because a lymphoproliferative disorder was suspected, a right hemicolectomy was done during which hard white studding was noted in the distal small bowel. Induration and a mass formation in the terminal ileum and the cecum were also seen. Histopathology showed necrotizing granulomas. Stain for acid-fast bacilli was negative. The strong suspicion for TB prompted us to obtain a chest computed tomography scan, which showed calcified perivascular and left hilar lymph nodes reflecting prior granulomatous disease. QuantiFERON-TB Gold In-Tube Test was positive. Treatment with standard anti-TB regimen was initiated. Two weeks later, cultures from intestinal tissue grew Mycobacterium tuberculosis. The patient reported a complete resolution of his symptoms at 3-month follow-up. Conclusions: Chest computed tomography scan and interferon. release assays in conjunction with TST and chest radiograph may improve the detection of latent TB in transplant candidates. Combining these tests to diagnose latent TB is a strategy that needs to be evaluated in future studies.
Background Locoregional therapy with curative intent (CLRT) followed by salvage liver transplantation (SLT) in case of hepatocellular carcinoma (HCC) recurrence is an alternative to primary liver transplantation (LT) in selected patients with HCC.Methods We performed a systematic review and meta-analysis of studies comparing the survival of patients treated with CLRT versus LT, stratified by the stage of liver disease, extent of cancer, and whether SLT was offered or not.Results We included 48 studies involving 9835 patients (5736 patients with CLRT and 4119 patients with primary LT). Five-year overall survival (OS) and disease-free survival (DFS) was worse for all categories of CLRT combined, than for primary LT (odds ratio [OR] for OS, 0.59; 95% confidence interval [CI], 0.48-0.71; P < 0.01). However, 5-year OS for CLRT and primary LT was not significantly different among patients with (i) Child-A cirrhosis and (ii) single HCC lesion, although DFS was worse. When SLT was offered after CLRT, intention-to-treat analysis showed no significant difference in 5-year OS (OR, 1.0; 95% CI, 0.6-1.7) between CLRT-SLT and primary LT, though noninferiority could not be shown. Only 32.5% patients with HCC recurrence after CLRT actually received SLT, as the rest were not medically eligible. Thus, the DFS was worse with CLRT-SLT (OR, 0.31; 95% CI, 0.2-0.6) compared with LT.Conclusions CLRT-SLT may be offered as first-line therapy to patients with HCC and well-compensated cirrhosis instead of primary LT because it may lead to better utilization of donor liver. However, a large proportion of patients with HCC recurrence after CLRT may not be candidates for SLT.
52 year old gentleman with hepatitis B received Liver Transplantation (LT) for decompensated cirrhosis. He had negative Tuberculin skin test (TST) and normal chest Xray on pre-LT screening. No known tuberculosis(TB) contacts. 2 years post-LT he presented with 8 weeks of abdominal pain, intermittent hematochezia and weight loss. He was on tacrolimus, cellcept and adefovir. Stool studies were negative. Serum CMV PCR was negative. CT scan showed thickening of ascending colon, cecum, appendix and terminal ileum(TI) with mesenteric stranding. Colonoscopy showed 50% circumferential ulcerated, polypoid mass at the junction of the cecum and ileocecal valve. Biopsy revealed fragments of inflammatorytype polyp with ulceration without dysplasia or malignancy. The cause of ulceration was histologically indeterminant. Immunostains for CMV, HSV and AFB were negative. Due to concern for lymphoproliferative disease, a right hemicolectomy was performed during which hard white studding throughout the distal small bowel, induration, and mass like conglomeration of the TI and the cecum were noted. Frozen section revealed granulomas. Histopathology revealed extensive transmural enterocolitis and necrotizing granulomas in the ileum, colon, appendix and several regional lymph nodes. Stains for AFB and fungi were negative. CT chest showed few small calcified perivascular and left hilar lymph nodes reflecting prior granulomatous disease. He was started on 4 drug anti-TB regimen. Two weeks later, tissue cultures grew acid fast bacilli that were identified as mycobacterium tuberculosis with DNA probe. At 3 month follow up, patient reported complete resolution of symptoms. LT recipients are screened for latent TB prior to LT with TST, interferon-γ release assays and chest X ray. TST has poor sensitivity due to anergy in cirrhotics. A number of patients with advanced cirrhosis have indeterminate test results with Quantiferon gold. History of prior exposure to TB, though important, has low sensitivity. One study showed improved detection of latent TB infection using chest CT scan. CT scan may not be feasible or cost-effective in all patients, but it could be considered in patients at high risk for latent TB (endemic regions, incarceration). Diagnosing TB reactivation post-LT is challenging. Quantiferon gold test requires adequate activation of T cells by TB antigens, which may be diminished post-LT. Hence, a high index of suspicion is needed to identify TB reactivation post-LT.Figure 1Figure 2
Introduction: Primary Liver Transplantation(LT) is the most common treatment modality in patients with Hepatocellular carcinoma(HCC) within Milan criteria. Loco-regional therapy(LRT) with curative intent- Surgical resection(SR), and Radiofrequency ablation(RFA), are alternate treatment strategies in selected patients with HCC. Salvage liver transplant(SLT) can be offered for selected patients with HCC recurrence following LRT. The survival outcome of patients with HCC undergoing LRT followed by SLT, as compared to primary LT is unclear. We aimed to compare the survival outcome of patients with HCC who underwent LRT with and without SLT as compared to patients who underwent LT. Methods: We performed a systematic review of the literature for studies investigating the survival outcome of patients with LRT including SLT as compared to LT. We recorded data on the characteristics of HCC, total number of patients, method of LRT used, presence or absence of cirrhosis, overall and disease-free survival rate, recurrence rate and median follow up. Revman version 5.2 was used to perform the meta-analysis using the random effects model. The studies were stratified into various groups to compare the survival outcomes between LRT and LT in each group. We used funnel plots to evaluate for publication bias. Results: 48 studies involving 9835 patients were included in the meta-analysis. LRT was inferior to LT with respect to both overall survival(OS) (Odds ratio (OR)0.69, Confidence interval (CI):0.56-0.85, 3 year; OR 0.59 (CI:0.48-0.71), 5 year) and disease free survival(DFS) (OR 0.30, CI:0.22-0.41, 5 year). Among patients with Child A cirrhosis, LRT was comparable to LT with respect to OS (OR: 0.8, CI:0.5-1.3, 3 year; OR 0.7, CI:0.4-1.4, 5 year) but was inferior to LT with respect to DFS (OR 0.3, CI:0.1-0.6, 5 year). SLT was comparable to LT with respect to both OS (OR 0.9, CI:0.5-1.4, 3 year; OR 0.8, CI:0.6-1.2, 5 year) and DFS (OR 0.7, CI:0.3-1.7). LRT followed by SLT (intention to treat) was comparable to LT with respect to OS at 5 year, OR 0.97, CI:0.5-1.7) but was inferior to LT with respect to DFS at 5 year (OR:0.4,CI:0.2-0.8). Conclusion: LRT followed by SLT seems to have a comparable overall survival to primary LT in patients with HCC. In patients who are candidates for curative LRT such as surgical resection or RFA, offering LRT with SLT instead of primary LT may increase the availability of donor livers to patients with advanced cirrhosis and may lead to an overall better utilization of donor livers.Table 1: Summary of meta-analysis of overall survival (OS) and disease-free survival (DFS) of patients with HCC undergoing Locoregional therapy (LRT) & Salvage Liver Transplant (SLT) vs. Liver Transplantation (LT)Figure 1Figure 2Figure 3