A case of Moyamoya disease complicated by aHUS in a patient with a history of marginal zone B-cell lymphoma: lightning strikes thrice?

Atypical haemolytic uraemic syndrome (aHUS) is a rare, acquired thrombotic microangiopathy, mediated by complement activation, in very sick patients. Moyamoya is similarly a rare disease in which stenosis or occlusion of segment(s) of the anterior cerebral circulation leads to the formation of many thin collaterals. Other reports have described an association between HUS and Moyamoya disease in the paediatric population. However, this case study presents the exceptionally rare presentation of an adult with aHUS and Moyamoya disease in a patient who was treated with rituximab for marginal zone B-cell lymphoma.

Opioid, alcohol, and cannabis misuse among patients with hidradenitis suppurativa: A population-based analysis in the United States.

BACKGROUND: Patients with hidradenitis suppurativa (HS) experience chronic pain and have significant physical, emotional, and psychological disease impact. These patients may be at risk for substance abuse. OBJECTIVE: To evaluate substance use disorder (SUD) among patients with HS in the United States. METHODS: This cross-sectional analysis of adult HS patients (n = 32,625) identified using electronic health records data was pulled from a population-based sample of >50 million patients. RESULTS: The prevalence of SUD among patients with HS was 4.0% (1315/32,625) compared to 2.0% (195,260/9,581,640) for patients without HS (P < .001). The most common forms of substance misuse among HS patients were alcohol (630/1315, 47.9% of SUD cases), followed by opioids (430/1315, 32.7% of SUD cases) and cannabis (430/1315, 29.7% of SUD cases). Patients with HS had 1.50 (95% confidence interval 1.42-1.59) times the adjusted odds of SUD compared to patients without HS. Patients with HS had significantly greater odds of SUD across demographic subgroups. The association between HS and SUD was generally stronger for patients 45 to 64 years of age, nonwhites, privately insured, and those without depressive or anxiety disorder. LIMITATIONS: SUD may not be accurately diagnosed. CONCLUSION: Patients with HS have higher odds of SUD and may benefit from periodic screening for substance abuse.

Validation of a Case-Finding Algorithm for Hidradenitis Suppurativa Using Administrative Coding from a Clinical Database.

BACKGROUND: Requisite to the application of clinical databases for observational research in hidradenitis suppurativa (HS) is the identification of an accurate case cohort. OBJECTIVE: To assess the validity of utilizing administrative codes to establish the HS cohort from a large clinical database. METHODS: In this retrospective study using chart review as the reference standard, we calculated several estimates of the diagnostic accuracy of at least 1 ICD-9 code for HS. RESULTS: Estimates of the diagnostic accuracy of at least 1 ICD-9 code for HS include sensitivity 100% (95% CI 98-100), specificity 83% (95% CI 77-88), positive predictive value 79% (95% CI 72-85), negative predictive value 100% (95% CI 98-100), accuracy 90% (95% CI 86-93), and kappa statistic 79% (95% CI 73-86). CONCLUSION: The case-finding algorithm employing at least 1 ICD-9 code for HS provides balance in achieving accuracy and adequate power, both necessary in the evaluation of a less common disease and its potential association with uncommon or even rare events.