Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis.

Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30-50% of patients with GPA, and may present as the initial presentation. Case Presentation: We describe two patients who presented with multiple deep, large, nonhealing skin ulcers postoperatively with purulent drainage and fever. Both patients were diagnosed with GPA after an extensive evaluation, including histopathology. Infectious, connective tissue disease and malignant etiologies were excluded. Their cANCA and PR3-ANCA levels were positive. Patient 2 was diagnosed early and recovered well after treatment with corticosteroids and rituximab; however, Patient 1 had a poor prognosis due to a long disease course. Conclusions: Diseases with multiple deep, large skin ulcers and fever can be infectious or noninfectious. Atypical manifestations may lead to missed diagnosis and misdiagnosis. GPA may initially present in a localized form before progressing to a generalized disease. The two cases we have highlighted will prompt clinicians to nevertheless call for a low threshold for diagnosis.

Shear wave elastography may be sensitive and more precise than transient elastography in predicting significant fibrosis.

BACKGROUND: Noninvasive measurements including transient elastography (TE) and two-dimensional shear wave elastography (SWE) have been used clinically instead of liver biopsy for regular assessment of liver fibrosis in chronic hepatitis B (CHB) patients. AIM: To investigate the diagnostic efficiency of SWE compared to TE by assessing independent influencing factors and performance for diagnosing significant fibrosis based on our cohort of treatment-naive CHB patients. METHODS: Fifty-four treatment-naive CHB patients who underwent liver biopsy to determine whether to initiate antiviral therapy were enrolled. SWE, TE, serum tests and liver biopsy were performed for all participants. The fibrosis-4 and aspartate aminotransferase to platelet ratio index scores were also calculated. Potential independent influencing factors on SWE and TE values were analyzed. Based on liver pathology results, the agreement and correlation were determined, and a comparison of the two methods was performed. RESULTS: There were 27 cases (50%) of mild fibrosis (F0-F2) and 27 (50%) cases of significant fibrosis (F3-F6); fibrosis was assessed with the Ishak scoring system. Multivariate linear regression analyses revealed that the fibrosis stage was the only factor that affected the SWE values (P < 0.001), whereas the total bilirubin level (P = 0.013) and fibrosis stage (P = 0.037) were independent factors that affected TE values. Orthogonal partial least squares discriminant analysis showed that the number of independent factors (VIP > 1) was higher for TE than SWE. Bland-Altman analysis showed satisfactory agreement between liver stiffness measurements (LSMs) of SWE and TE. Both SWE and TE could significantly discriminate significant fibrosis from mild fibrosis (P < 0.001). SWE exhibited a higher correlation with LSMs of liver fibrosis than TE (r = 0.65 and 0.50, P < 0.001). The diagnostic performance of SWE was better than that of TE for significant fibrosis (F > 2). The areas under the receiver operating characteristic curves of SWE and TE were 0.786 and 0.714, respectively. The optimal LSM cutoff values of SWE and TE were 9.05 kPa and 8.15 kPa, respectively. CONCLUSION: Compared to the TE value, the SWE value was less affected by other factors. SWE may be more sensitive and precise than TE in predicting significant fibrosis (> F2) in CHB patients.

Treatment of primary biliary cholangitis with ursodeoxycholic acid, prednisolone and immunosuppressants in patients not responding to ursodeoxycholic acid alone and the prognostic indicators.

BACKGROUND AND AIM: We reviewed the medical records of primary biliary cholangitis patients who were diagnosed by liver biopsy and treated with the corresponding treatment. We evaluated the therapeutic effect and long-term prognostic indicators. METHODS: This observational cohort study enrolled 80 eligible patients diagnosed by liver biopsy between December 2013 and December 2018 in our department. UDCA monotherapy or UDCA added to prednisolone and immunosuppressant triple therapy was prescribed to patients. We analyzed and compared the demographic characteristics, biochemistry profiles, immune parameters, and noninvasive liver fibrosis assessments at baseline as well as the treatment efficacy, long-term outcomes and adverse effects at baseline and at each visit between the two groups. The indicators that could affect prognosis were assessed. RESULTS: Thirty-eight primary biliary cholangitis patients received UDCA monotherapy (group A), and another 42 patients received UDCA, prednisolone and immunosuppressant triple therapy (group B). After therapy, all patients showed significant improvements in liver biochemical parameters, immune indicators, and noninvasive fibrosis indicators (Fibrosis-4 (FIB-4), aspartate aminotransferase-to-platelet ratio index (APRI)), all P values<0.0001. The Mayo score also decreased significantly after treatment (P=0.022). Triple therapy was more effective, and there was a significant difference between the two groups. In addition, multivariate analysis showed that anti-gp210 antibody positivity; antimitochondrial antibody (AMA) negativity; high alkaline phosphatase (ALP), total bilirubin (TBIL) and globulin levels; and a severe degree of fibrosis at baseline were independent predictors of a poor prognosis. CONCLUSIONS: Triple therapy was a treatment option for UDCA-refractory PBC patients. Anti-gp210 antibody positivity; AMA negativity; high ALP, TBIL and globulin levels; and a severe degree of fibrosis at baseline were associated with a poor prognosis.

Gilbert syndrome combined with prolonged jaundice caused by contrast agent: Case report.

This case highlights a patient with Gilbert syndrome who underwent endoscopic retrograde cholangiopancreatography (ERCP) with removal of bile duct stones, who then experienced an unexplained increase in bilirubin, with total bilirubin (TBIL) levels increasing from 159.5 µmol/L to 396.2 µmol/L and to a maximum of 502.8 µmol/L after 9 d. Following the decrease in the TBIL level, enhanced magnetic resonance cholangiopancreatography (MRCP) was performed to exclude any possible remaining choledocholithiasis. Nevertheless, the serum bilirubin level increased again, with TBIL levels rising from 455.7 µmol/L to 594.8 µmol/L and a maximum level of 660.3 µmol/L with no remaining bile duct stones. A liver biopsy showed severe bile duct cholestasis with no inflammation. Based on the exclusion of other potential causes of hyperbilirubinemia and the fact that both instances of increased bilirubin occurred after ERCP and MRCP, the contrast agents iopromide and gadoterate meglumine were suspected to be the causes of the hyperbilirubinemia. As of the writing of this report, the patient's bilirubin levels have spontaneously returned to baseline levels. In summary, ERCP and MRCP utilizing the contrast agents iopromide and gadoterate meglumine may possibly induce prolonged hyperbilirubinemia.