Journal Club August 2022

Birt-Hogg-Dubé syndrome in apparent primary spontaneous pneumothorax patients; results and recommendations for clinical practice. 

Sriram JD, et al.

BMC Pulm Med.

PMID: 36028846

This cohort from the Netherlands examined the prevalence of BHD in patients who had previously presented with apparent primary spontaneous pneumothorax to their centre. 475 apparent spontaneous pneumothorax patients met their inclusion criteria and were sent a questionnaire. Ultimately, just 88 patients were invited for genetic testing. They also performed CT chests in those who had not had one done previously.

From the 88 patients included, 3 (3.4%) had pathogenic variants in FLCN were detected in keeping with BHD. (c.1301-7_1304del; 1323delinsGA (twice), and c.1408_1418del). All 3 patients were female and 2 were directly related.

All patients with BHD had multiple cysts (n = 3, 3 and 9 respectively) and fibrofolliculomas. The majority of cysts were located in the basal lung fields below the level of the carina.

The prevalence of BHD is lower than previously reported series of primary pneumothoraces (Chinese cohorts). The authors postulate that the denominator might be greater in their cohort, as Dutch people are generally much taller increasing their risk of primary idiopathic pneumothorax. 

Delayed diagnosis of Birt-Hogg-Dubé syndrome might be aggravated by gender bias. 

Steinlein OK, et al.

EClinicalMedicine.

PMID: 35875814 Free PMC article.

A well written case series of 158 patients diagnosed with BHD at a tertiary referral centre in Germany. Patients were highlighted to the service for a variety of reasons: The reasons for diagnosis were a family history for BHDS (n-76), fibrofolliculoma (n-40), pneumothorax (n-21), RCC (n-19), and renal adenomas (n-1). Mean age at diagnosis was around 33.

Most striking was the delays to diagnosis depending on the presenting symptom and gender. Patients presenting with a pneumothorax had on average an 18year delay before the diagnosis of BHD was made. It is important given that 4 patients developed a meta-synchronous RCC whilst the diagnosis of BHD was missed.

The impact of the COVID-19 pandemic on pleural infection incidence: a UK multicentre retrospective analysis. 

Bedawi EO,  et al.

ERJ Open Res.

PMID: 35919861 

Using data collected from screening logs of the multicentre UK MIST3 trial, Behawi and colleagues have shown that the reduction in pleural infection admissions during the 1st and 2nd waves of COVID-19 was not just anecdote.

Two comparative periods were chosen as March 2020 to February 2021 (post-COVID), to represent the study cohort, against the same period before the COVID-19 pandemic (March 2019 to February 2020; pre-COVID) as a control cohort.

In the 1-year pre-COVID period, 184 new cases of pleural infection were identified across the five participating centres versus 124 new cases in the 1-year period following the start of the pandemic (a 32.61% reduction).

The authors propose a number of potential reasons including reduced community transmission of viruses due to social distancing, use of personal protective equipment in both community and healthcare settings and patient hesitancy to attend hospital during peaks of COVID-19.

NICE guidelines on nivolumab plus ipilimumab for untreated, unresectable malignant pleural mesothelioma. 

Adler AI, et al.

Lancet Respir Med.

PMID: 35988573 

On Aug 17, 2022, the National Institute for Health and Care Excellence (NICE) published guidance recommending nivolumab plus ipilimumab as an option for treating previously untreated, unresectable malignant pleural mesothelioma in adults with an Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1.

Spontaneous bacterial empyema in cirrhosis: A systematic review and meta-analysis. 

Reiche W, et al.

World J Hepatol.

PMID: 35978675 Free PMC article.

Effects of Different Treatment Regimens on Primary Spontaneous Pneumothorax: A Systematic Review and Network Meta-Analysis. 

Muhetaer M, et al.

PMID: 36002271

Comparison of clinical utility between digital and analog drainage systems in patients with spontaneous pneumothorax. 

Yagi S, et al.

Respir Investig.

PMID: 35965216