Comparison between the ATS/ERS/JRS/ALAT criteria of 2011 and 2018 for Usual Interstitial Pneumonia on HRCT: a cross-sectional study.

OBJECTIVES: To determine whether the revised 2018 ATS/ERS/JRS/ALAT radiological criteria for usual interstitial pneumonia (UIP) provide better diagnostic agreement compared to the 2011 guidelines. METHODS: Cohort for this cross-sectional study (single center, nonacademic) was recruited from a multidisciplinary team discussion (MDD) from July 2010 until November 2018, with clinical suspicion of fibrosing interstitial lung disease (n= 325). Exclusion criteria were technical HRCT issues, known connective tissue disease (rheumatoid arthritis, systemic sclerosis, poly-or dermatomyositis), exposure to pulmonary toxins or lack of working diagnosis after MDD. Four readers with varying degrees in HRCT interpretation independently categorized 192 HRCTs, according to both the previous and current ATS/ERS/JRS/ALAT radiological criteria. An inter-rater variability analysis (Gwet's second-order agreement coefficient, AC2) was performed. RESULTS: The resulting Gwet's AC2 for the 2011 and 2018 ATS/ERS/JRS/ALAT radiological criteria is 0.62 (±0.05) and 0.65 (±0.05), respectively. We report only minor differences in agreement level among the readers. Distribution according to the 2011 guidelines is as follows: 57.3% 'UIP pattern', 24% 'possible UIP pattern', 18.8% 'inconsistent with UIP pattern' and for the 2018 guidelines: 59.6% 'UIP', 14.5% 'probable UIP', 15.9% 'indeterminate for UIP' and 10% 'alternative diagnosis'. CONCLUSIONS: No statistically significant higher degree of diagnostic agreement is observed when applying the revised 2018 ATS/ERS/JRS/ALAT radiological criteria for UIP compared to those of 2011. The inter-rater variability for categorizing the HRCT patterns is moderate for both classification systems, independent of experience in HRCT interpretation. The major advantage of the current guidelines is the better subdivision in the categories with a lower diagnostic certainty for UIP. ADVANCES IN KNOWLEDGE: - In 2018, a revision of the 2011 ATS/ERS/JRS/ALAT radiological criteria for UIP was published, part of diagnostic guidelines for idiopathic pulmonary fibrosis.- The inter-rater agreement among radiologist is moderate for both classification systems, without a significantly higher degree of agreement when applying the revised radiological criteria.

Nivolumab and anti-HCV activity, a case report.

Exhaustion of antigen-specific T-cells in order to escape immune destruction is frequently seen in chronic viral infection and different types of cancer. Blockade of overexpressed negative co-stimulatory pathways, a process known as immune checkpoint modulation, is a promising novel therapy that could improve the treatment of liver diseases with features of T cell exhaustion. We present a case of a 54-year-old hepatitis C virus (HCV) positive patient with an acute flare of hepatitis during nivolumab treatment for a stage IV lung carcinoma, an anti-programmed death-1 (PD-1) immunotherapy. Retrospective testing of HCV RNA documented infection more than 6 months ago. Nivolumab treatment was associated with an alanine aminotransferase (ALT) flare reaching a peak value of 663 U/L, along with bilirubin levels of 0.74 mg/dL and no signs of coagulopathy. The assumption of a nivolumab-associated autoimmune hepatitis led to the interruption of the immune checkpoint inhibitor treatment. However, a subsequent 1-log decrease of HCV RNA load was noticed, which raised the possibility of an immune reconstitution against the HCV-infected hepatocytes with cell lysis. Liver biopsy specimen demonstrated no evidence for autoimmune liver disease or fibrosis. Clinical evolution was favorable and serum transaminases returned to normal levels and HCV RNA load increased to baseline values following nivolumab cessation. The current case suggests an anti-HCV activity of anti-PD-1 treatment in the setting of concomitant HCV viremia and lung carcinoma.

Cochlear Erosion due to a Facial Nerve Schwannoma.

Facial nerve schwannomas are rare benign neoplasms. We report a case of a 60-year-old woman who initially presented with vestibular complaints. Magnetic resonance imaging (MRI) revealed a facial nerve schwannoma centered on the right geniculate ganglion extending in the labyrinthine segment. The patient consulted again after 2 months because she developed a sudden and severe right-sided sensorineural hearing loss. MRI showed no progression or pathological enhancement in the membranous labyrinth. A cone beam computed tomography (CT) of the temporal bone was performed and revealed a large erosion at the region of the geniculate ganglion in open communication with the middle turn of the cochlea. This case report demonstrates the importance of CT in facial nerve schwannomas for evaluating the impact on the surrounding structures.

5-Year survival after splenectomy in a patient with recurrent cutaneous melanoma: a case report.

OBJECTIVE: Splenic metastases of melanoma are uncommon and generally part of a disseminated disease with a poor prognosis. In case of solitary visceral metastasis, surgical resection often remains an effective treatment. This case describes a long-term survival after splenectomy for a recurrent melanoma. METHODS: We present a 55-year-old female with a solitary splenic metastasis. Her medical history revealed a melanoma of the foot in 2007 and an in-transit metastasis in 2011. A PET-CT revealed in 2012 the presence of a small aspecific lesion in the spleen. Close follow-up was chosen. Since PET-CT and MRI showed a progressive lesion, a laparoscopic splenectomy was performed. RESULTS: Histological report confirmed the diagnosis of a metastatic lesion. Sixty months after the first radiographic evidence of the splenic metastasis, follow-up revealed no signs of residual disease. CONCLUSIONS: Although current systemic approaches demonstrate to provide clinically important benefit, patients with oligometastatic disease should be evaluated for surgical metastasectomy. A laparoscopic approach for splenic metastasis is shown to be a safe treatment. This disease-free survival of 60 months in a patient with a history of an in-transit metastasis has not been published before. A splenectomy for a solitary metastasis could prolong the survival, even in a recurrent melanoma.

Palpitations, headache and night sweats caused by a retroperitoneal mass: case report and short review.

A 31-year-old West-African female attended our emergency department presenting with palpitations, headache, fatigue and night sweats during the last 2 weeks. Clinical examination revealed tachycardia and a painful, palpable infraumbilical mass. Ultrasound examination of the abdomen showed a smoothly rounded soft-tissue mass with a diameter of 5 cm. On contrast-enhanced CT, a prevertebral mass with intense contrast enhancement was seen, located caudal to the aortic bifurcation. On PET-CT, there were no distant 18F-FDG-avid locoregional nodes or masses. A tumourectomy was successfully performed, during which manipulation of the retroperitoneal tumour triggered a sharp rise in blood pressure. Histological analysis confirmed the diagnosis of a paraganglioma. The clinical complaints of headache, paroxysmal palpitations and night sweats disappeared postoperatively. This case is a classic presentation of a paraganglioma occurring in the organs of Zuckerkandl, a collection of paraganglia. The diagnosis should be suspected in the presence of a heterogeneous, hypervascular mass in the retroperitoneum and typical clinical symptoms of hypertension, headache and palpitations. Treatment involves surgical resection, after accurate preoperative management. Genetic counselling is required, allowing a personal and genotype-based follow-up.

Mepacrine as successful monotherapy for refractory Jessner-Kanof disease: still an important drug in the dermatologic armamentarium.

INTRODUCTION: Jessner-Kanof disease (JKD), a lymphocytic infiltration of the skin, can be difficult to treat. Mepacrine (quinacrine), an anti-malarial less available in Belgium, may be beneficial. PATIENTS AND METHODS: Two female patients with biopsy-proven and therapy-resistant JKD, not responding to topical and systemic corticosteroids, (hydroxy-)chloroquine and/or dapsone, were treated with mepacrine 100 mg daily. RESULTS: In both patients an amelioration was observed during the first month of treatment, and clinical remission was obtained by the fourth month, without any side-effects. In both cases, the dose could be tapered to three times weekly. DISCUSSION: JKD is strongly related to lupus erythematosus (tumidus), and although spontaneous remissions may occur, it is notoriously difficult to treat. Mepacrine may be initiated as an add-on therapy to (hydroxy-)chloroquine, but also as monotherapy. A dose of 100 mg a day, tapered to weekly doses once remission is obtained, seems feasible. Except for (mild) yellow skin discoloration, the drug has few side-effects, and offers the advantage of not displaying retinal toxicity. CONCLUSION: Mepacrine is still a useful and safe drug for treating cutaneous lupus erythematosus and related skin conditions, such as refractory JKD in particular. Its future availability, also in Belgium, is therefore important.

Diffusion Kurtosis Imaging: A Possible MRI Biomarker for AD Diagnosis?

The purpose of this explorative study was to investigate whether diffusion tensor imaging (DTI) and diffusion kurtosis imaging (DKI) parameter changes are reliable measures of white matter integrity changes in Alzheimer's disease (AD) patients using a whole brain voxel-based analysis (VBA). Therefore, age- and gender-matched patients with mild cognitive impairment (MCI) due to AD (n = 18), dementia due to AD (n = 19), and age-matched cognitively healthy controls (n = 14) were prospectively included. The magnetic resonance imaging protocol included routine structural brain imaging and DKI. Datasets were transformed to a population-specific atlas space. Groups were compared using VBA. Differences in diffusion and mean kurtosis measures between MCI and AD patients and controls were shown, and were mainly found in the splenium of the corpus callosum and the corona radiata. Hence, DTI and DKI parameter changes are suggestive of white matter changes in AD.