The SPEC score-A quantifiable CT scoring system for primary ciliary dyskinesia.

BACKGROUND: Structural lung changes seen on computed tomography (CT) scans in persons with primary ciliary dyskinesia (pwPCD) are currently described using cystic fibrosis (CF) derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD-derived scoring system. METHODS: Chest CT scans from 30 pwPCD, were described for structural changes including bronchiectasis, bronchial wall thickening, mucous plugging, atelectasis, air trapping, and interlobar septal thickening and, additionally, changes previously described as being frequent in pwPCD including extensive tree-in-bud pattern of mucous plugging, bronchoceles or nodules, thickening of interlobar and interlobular septa and whole lobe atelectasis. Based on these findings a novel and unique scoring system, the Specific PCD Evaluation by CT (SPEC) score was constructed. Scans were then re-scored using the SPEC score and results compared to corresponding measurements of lung function to assess structure-function correlation. RESULTS: Total SPEC scores ranged from 0 to 60 (max possible score 90). There was a strong negative correlation between the SPEC score (SPEC) and forced vital capacity (FVC), forced expiratory volume over 1 s (FEV1 ) and FEV1 /FVC ratio (-r = .784, -.865, -.872 respectively). CONCLUSIONS: Using PCD-derived data we describe the construct of a PCD-specific score for assessing lung structural damage on CT scans, the SPEC score. A strong correlation between the SPEC score and PFT variables was identified. The SPEC score holds the potential for describing longitudinal changes in CT scans and assessing the efficacy of interventive therapies in patients with PCD.

Management of cutaneous side effects of inflammatory bowel disease therapy: A dermatologic viewpoint.

Medications used in the treatment of inflammatory bowel disease cause a wide range of dermatologic side effects, and minimal guidance exists on how to manage them. The intention of this review article is to summarize common dermatologic adverse reactions related to inflammatory bowel disease therapy and to provide evidence-based guidance on management. We conducted a scoping review using PubMed and Google Scholar to identify studies reporting clinical information on dermatologic side effects of medications used in the treatment of inflammatory bowel disease. The most commonly reported dermatological adverse effects from inflammatory bowel disease therapy were cutaneous malignancy and cutaneous infections. Thiopurines, methotrexate, tumor necrosis factor (TNF) inhibitors, interleukin (IL)-12/23 inhibitors, and integrin inhibitors can be continued if nonmelanoma skin cancer arises during therapy and the malignancy should be surgically excised. TNF inhibitors and IL-12/23 inhibitors can be continued in the setting of stage I surgically resectable melanoma but should be discontinued in advanced melanoma. For complicated cutaneous bacterial infections, methotrexate and TNF inhibitors should be halted, and IV antibiotics should be administered. Complicated herpes zoster infection warrants discontinuation of TNF inhibitors, whereas IL-12/23 and JAK inhibitors can be continued. Inflammatory bowel disease therapies are associated with several dermatological adverse effects, and management options vary by agent. Certain agents may require discontinuation in the setting of nonmelanoma skin cancer, melanoma, and cutaneous infections. Many other dermatological adverse effects from inflammatory bowel disease therapy require specialized management or referral to dermatology.

Social Media Use for Inflammatory Bowel Disease in a Rural Appalachian Population.

Background: Social media use is prevalent in our society and has become widely used in the health care community. Inflammatory bowel disease (IBD) patients constitute one of the patient populations that benefit from social media use to obtain information on their diseases. West Virginia (WV) is a rural Appalachian state with barriers to internet access and health care and we examined the role that social media plays in patients' lives in this state, which could be reflective of other rural states. Methods: Our patient population consisted of patients, 18-65 years old, who live in WV with a diagnosis of IBD. A 17-question survey was sent to 2,131 patients over a course of 4 weeks through an application called REDCap. Results: We received 624 responses with a 29% response rate. Approximately 30% of patients reported that they used Facebook for IBD-related information, while 4.3% used Instagram. While most (92%) patients preferred information coming from their physician, the majority judged information from the internet to be reliable (39.3%) or neutral (44.9%). Most patients believed that social media had no impact on their disease management (67%), while 30.3% believed it had a positive impact. Almost 45% of patients stated that they wished their physician had a social media account for IBD. Conclusions: Our study shows that patients are interested in obtaining health-related information from social media resources. As physicians, it is our job to point them in the right direction to be able to find reliable information.