Diagnosis of acute intestinal GVHD (aGVHD) following allogeneic hematopoietic cell transplantation is based on clinical symptoms and histological lesions. This retrospective analysis aimed to validate the ‘Freiburg Criteria’ for the endoscopic grading of intestinal aGVHD. Grade 1: no clear-cut criteria; grade 2: spotted erythema; grade 3: aphthous lesions; and grade 4: confluent defects, ulcers, denudation of the mucosa. Having excluded patients with infectious diarrhea, we evaluated 175 consecutive patients between January 2001 and June 2009. Setting a cutoff between grade 1 (no change in therapy) and grade 2 (intensification of immunosuppression), macroscopy had a sensitivity of 89.2% (95% confidence interval (CI): 80.4–94.9%), a specificity of 79.4% (95% CI: 69.6–87.1%), a positive-predictive value of 79.6% (95% CI: 70.0–87.2%) and a negative-predictive value of 89.0% (95% CI: 80.2–94.9%). In all, 20% of patients with aGVHD in the lower gastrointestinal tract (GIT) had lesions only in the terminal ileum. In all patients with aGVHD 2 of the upper GIT, typical lesions were also found in the lower GIT. Ileo-colonoscopy showed the highest diagnostic yield for aGVHD. In conclusion, the ‘Freiburg Criteria’ for macroscopic diagnosis of intestinal aGVHD provide high accuracy for identifying aGVHD 2.

Background There is a paucity of data on fertility or pregnancy in patients with primary sclerosing cholangitis (PSC). Objective To assess fertility in PSC by comparing the number of children in a large cohort of PSC patients to healthy controls and to investigate the outcome of pregnancy, as well as the influence of pregnancy on the disease course. Design Case series. Setting Germany. Participants 229 PSC patients and 569 healthy controls were evaluated for the number of children. 17 patients with PSC and at least one pregnancy, or who received a diagnosis of PSC within 6 months after delivery, were included in the more detailed analysis. Main outcome measures Number of children per patient and control; disease activity during pregnancy and after delivery including maternal complications; long-term development of live births, fetal loss rate and the influence of medication on fetal and maternal outcome. Results Fertility did not seem to be reduced in PSC since the number of children did not differ between PSC patients and healthy controls. 25 pregnancies in 17 female PSC patients (median age at conception 31 years) were investigated in detail. An increase in liver enzymes was documented during five pregnancies (20%) and eight times (32%) post-partum. There were no serious maternal complications. All 21 live births presented with a normal perinatal and postnatal development over a median observation time of 50 months. Two pregnancies were delivered pre-term and four fetal losses occurred early in pregnancy (<12 wk). Continuation of treatment with ursodeoxycholic acid (15/21) or azathioprine (2/21) had no negative effects on pregnancy outcome. Conclusions Fertility does not seem to be reduced in patients with PSC, who are able to deliver healthy children without an apparent increase in risk for mother or child.

Summary: Background: Despite advances in immunosuppressive therapy, up to 10% of patients with severe Crohn's disease (CD) remain refractory to conventional treatment. Limited evidence from pilot trials suggests that high‐dose immunosuppression and autologous peripheral blood stem cell transplantation (autoPBSCT) may induce remission in these patients, but there is substantial controversy regarding the safety and efficacy of this approach.Aim: To address this issue, a monocentre phase I/II trial of autoPBSCT was performed in patients with refractory CD in our hospital.Methods: Here, we report on the outcome of 12 patients with refractory CD treated with autoPBSCT. Briefly, CD34⁺‐selected PBSCs were harvested after mobilisation therapy with cyclophosphamide and granulocyte‐colony stimulating factor. Later, immunoablative conditioning therapy with high‐dose cyclophosphamide followed by autoPBSCT was applied and clinical and endoscopic responses were analysed after a mean follow‐up of 3.1 years (range 0.5–10.3 years).Results: PBSC harvest following mobilisation chemotherapy was successful in 11/12 patients and resulted in a clinical and endoscopic improvement in 7/12 patients. Subsequent conditioning and autoPBSCT were performed in nine patients and were relatively well tolerated. Among those, five patients achieved a clinical and endoscopic remission within 6 months after autoPBSCT. However, relapses occurred in 7/9 patients during follow‐up, but disease activity could be controlled by low‐dose corticosteroids and conventional immunosuppressive therapy.Conclusion: Immunoablation by cyclophosphamide and autologous peripheral blood stem cell transplantation is safe and effective to induce remission of refractory Crohn's disease, and should be further evaluated in randomised controlled trials.

Purpose: To evaluate the feasibility of time‐resolved flow‐sensitive MRI for the three‐dimensional (3D) visualization and quantification of normal and pathological portal venous (PV) hemodynamics.Materials and Methods: Portal venous hemodynamics were evaluated in 18 healthy volunteers and 5 patients with liver cirrhosis. ECG‐ and adaptive respiratory navigator gated flow‐sensitive 4D MRI (time‐resolved 3D MRI with three‐directional velocity encoding) was performed on a 3 Tesla MR system (TRIO, Siemens, Germany). Qualitative flow analysis was achieved using 3D streamlines and time‐resolved particle traces originating from seven emitter planes precisely placed at anatomical landmarks in the PV system. Quantitative analysis included retrospective extraction of regional peak and mean velocities and vessel area. Results were compared with standard 2D flow‐sensitive MRI and to the reference standard Doppler ultrasound.Results: Qualitative flow analysis was successfully used in the entire PV system. Venous hemodynamics in all major branches in 17 of 18 volunteers and 3 of 5 patients were reliably depicted with good interobserver agreement (kappa = 0.62). Quantitative analysis revealed no significant differences and moderate agreement for peak velocities between 3D MR and 2D MRI (r = 0.46) and Doppler ultrasound (US) (r = 0.35) and for mean velocities between 3D and 2D MRI (r = 0.41). The PV area was significantly (P < 0.01) higher in 3D and 2D MRI compared with US.Conclusion: We successfully applied 3D MR velocity mapping in the PV system, providing a detailed qualitative and quantitative analysis of normal and pathological hemodynamics. J. Magn. Reson. Imaging 2010;32:466–475.

Objective: Lichen planus (LP) is a classic skin disease that can involve the skin, hair, and nails, as well as the oral and genital mucosa. Histopathology is characterized by a T-lymphocytic, lichenoid, and interface dermatitis. Multiple case reports and small case series have shown that LP can involve the esophagus. However, the diagnostic criteria, incidence, and best treatment options remain uncertain. This study aimed to refine the diagnostic criteria, estimate prevalence, and present an outlook on treatment options to prevent long-term sequelae. Patients and methods: Thirty-two consecutive patients with LP of the skin, hair, nails, oral mucosa, and/or genital mucosa underwent a comprehensive clinicopathologic assessment. Esophagogastroduodenoscopy was performed, and biopsies were evaluated histologically, immunohistochemically, and by direct immunofluorescence. Patients diagnosed with esophageal lichen planus (ELP) were followed up prospectively where possible. Results: In total, 20 of 32 patients had ELP. Ten of these 20 patients were classified as having proven ELP, with clear-cut endoscopically visible lesions; the other 10 were classified as having probable ELP. Eight of 10 patients with proven ELP were started on new or additional therapy because of esophageal findings. Treatment with a topical budesonide formulation or systemic corticosteroids was successful in most patients with proven ELP and reversed functional esophageal stenosis. Conclusion: ELP can be found in more than 50% of patients with proven mucocutaneous LP when clinical and pathologic findings are correlated carefully. Topical or systemic corticosteroids are the first-line therapy for ELP. Timely medical therapy seems to prevent scarring stenosis of the esophagus.