TL;DR – 800 patients with severe pneumonia admitted to the ICU randomized to 200mg daily hydrocortisone vs placebo; reduced mortality (6.2% vs 11.9%), higher ICU discharge, less intubation (18% vs 29.5%)! Note those with septic shock were excluded (probably because they would receive hydrocort 50q6 anyway?).
Especially beneficial in subgroup with high CRP (makes sense)
Last week, Anthony presented a patient with a reduction in DLCO and mild restrictive spirometric defect on PFTs CT scan showed questionable interstitial changes. We discussed further evaluation and management of ILAs.
How do we define Interstitial Lung Abnormalities (ILAs) (1)?
in patients for whom ILD is not clinically suspected
Mimickers of ILAs include dependent atelectasis, interstitial edema, aspiration (GGOs, TiB opacities) and others
Image: focal paraspinal fibrosis (arrow) in contact with spinal osteophytes, a mimicker of ILAs
How common are ILAs, and what is their significance?
Large population-based (MESA, AGES, FHS, Nagano) and smoking/lung ca. screening (ECLIPSE, NLST, COPDGene, MILD) cohorts found that ILAs are present in 4-10%of individuals screened.
Mean age of scan with ILA varies between 62-78
Progression to ILD observed in 20% at 2 years, and 45-65% at 4-6 years (depending on study, not all cohorts assessed for progression)
Mortality risk associations suggested in several studies, with RR between 1.3-2.7 (95% CI >1, depending on study, not all cohorts assessed for mortality)
Risk factors for ILAs are similar to ILD: increased age, male sex, tobacco smoke exposure
Table: clinical risk factors for ILD progression (2)
Can ILAs be further categorized, and what are implications of these categories?
Non-subpleural: usually non-progressive and not associated with increase in mortality
Subpleural non-fibrotic: sub pleural distribution associated with higher rates of mortality
Subpleural fibrotic: lower zone predominant, reticulation, traction bronchiectasis associated with 6x increased risk of progression
How should we manage patients with ILAs?
An algorithmic approach is described in the image below:
Takeaway points:
ILAs are (1) incidentally found (2) non-dependent (3) involving at least 5% of lung zone and (4) in patients for whom ILD is not suspected
ILAs are present in 4-10% of patients screened, and typically found in 6th/7th decade of life, and are associated with increased mortality (RR 1.3-2.7)
Progression is 20% at 2 years, 45-65% at 5 years
Risk of progression is higher in subpleural location and if reticulations/fibrosis are present
We wanted to spotlight the great work fellowship-graduate Dr. Justin Fiala has been doing with CHI-PAP, an initiative to evaluate and treat sleep-related concerns. CommunityHealth is a free clinic that has multidisciplinary care under one roof. Dr. Fiala has built an amazing program that takes advantage of refurbished home sleep testing devices and donated and recycled/refurbished sleep devices and equipment to bring comprehensive sleep health to the community.
Today in Pulmonary Report, the Emily Olson series continues with a case of cystic lung disease. Today, Emily described the management of a young woman with recurrent secondary spontaneous pneumothorax, found to have cystic lung disease.
Before we dive into today’s report, we encourage you to take this opportunity for some retrieval practice from prior NU PCCM blog posts:
For review, here are the blog posts associated with polls above
A case of cystic lung disease in patient with Sjogren’s syndrome from ILD Roundup last July.
Management of secondary spontaneous pneumothorax with delayed resolution (Leak for a week!) from Emily’s Morning Report last August
A case of secondary spontaneous pneumothorax from cystic lung disease from Ale’s Morning Report last September
Alright! Now that the knowledge is fresh, let’s dive in! First, we discussed definitional criteria and a differential diagnosis (1) of cystic lung disease:
Another outstanding quick reference on this topic is Nick Mark’s OnePager (2):
Emily outlined the pragmatic 5-step approach described by Raoof et al.
Further refinement of differential involves considering the apicobasilar gradient of illness:
Emily’s case presented a bit of a diagnostic dilemma – LAM was clinically suspected but VEGF testing performed and unrevealing. Patient ultimately proceeded to VATS resection of a peripheral cyst/bulla, but pathology was inconclusive. This brought up an important discussion point – does it really matter if we know that cause?
Revealing cause of cystic lung disease may have important management implications for our patients:
Genetic inheritance of cystic lung diseases and multi-system illnesses associated. Genetic counseling/sequencing may be indicated
Differential considerations for cystic lung diseases include metastatic malignancies and certain infectious processes with specific management
Dr. Russell also brought up the higher risk of malignancy in patients with Birt-Hogg-Dube (BHD) specifically – particularly colorectal cancer. Recall also from Ale’s morning report – 7x risk of RCC, screening with annual ultrasound in US. BHD, which follows an AD inheritance pattern, may be confirmed by genetic testing revealing a mutation in the FLCN gene, although testing may not be covered by insurance.
Dr. Singer also mentioned the NEJM study (3) that found sirolimus to be associated with stabilization of lung function and improved quality of life in patients with Lymphangioleiomyomatosis (LAM).
Especially beneficial in subgroup with high CRP (makes sense)
Thanks for an outstanding discussion, Emily!