NU PCCM Fellowship Blog | Category

Morning Report 3-1-23

Date March 1, 2023

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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.

First-year PCCM fellow Emily Olson (@EmilyOlsonMD)

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).

Some take-home points:

Thanks for an outstanding discussion, Emily!

Sources:

PMID: 31704148 / DOI: 10.1016/j.chest.2019.10.017
https://onepagericu.com/cystic-lung-disease
PMID: 21410393 / DOI: 10.1056/NEJMoa1100391

ILD Round Up 2-19-23

Date February 19, 2023

Posted by Tim Rowe

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In last week’s ILD conference, we discussed a patient with seropositive RA for whom RA-ILD was suspected.

First, a quick reminder of ILD patterns associated with rheumatoid arthritis (1) from July ILD Round Up:

Dr. Rishi Agrawal mentioned that this patient’s HRCT pattern was most consistent with UIP; however, given this patient’s history of connective tissue disease, “exuberant honeycombing” may be expected, and the lack of any honeycombing was somewhat atypical.

What characteristics are commonly associated with a UIP pattern in patients with CT-ILD, and how might they differ from UIP in a patient with IPF?

A 2018 study (2) looked at patients with UIP with either a diagnosis of IPF or CTD-ILD. Exuberant honeycombing was found in only 6% of IPF patients compared to 22% of patients with CTD-ILD.

While we’re on the subject…what is exuberant honeycombing?

Exuberant honeycombing (3) refers to extensive honeycomb-like cyst formation within the lungs comprising >70% of the fibrotic portions of the lungs.

Finally, we discussed the patient’s prior treatment regimen, which included methotrexate (MTX).

What is the likelihood of methotrexate-associated interstitial lung disease in patients with RA-ILD on methotrexate?

A case-control study (4) looking at the association between MTX exposure and ILD (n=1083 patients) found an inverse correlation between MTX exposure and RA-ILD (OR 0.46: 0.24-0.90, p=0.02). Another meta-analysis (5) of 30 studies with 15000 total patients found no association (RR 1.02: 0.73-1.44) for non-infectious adverse respiratory events with MTX use.

Dr. Carrie Richardson noted that many patients with more severe RA will have already failed therapy with methotrexate for their articular symptoms, which may confound anecdotal reports of ILD associated with MTX use.

Dr. Jane Dematte also mentioned the distinct entity of MTX associated hypersensitivity. This association is well-recognized, with frequency level 5 from Pneumotox profile corresponding to >200 reported cases in the literature.

The actual frequency of occurrence depends on definitional criteria (see figure below) but is likely <1%. Below, see the commonly used Carson Criteria for diagnosis of MTX associated pneumonitis (MTX-P).

Sources:

1. DOI: 10.1183/09059180.00008014 / PMID: 25726549

2. DOI: 10.2214/AJR.17.18384 / PMID: 29140119

3. DOI: 10.1016/j.ejro.2022.100419 / PMID: 35445144

4. DOI: 10.1183/13993003.00337-2020 / PMID: 32646919

5. DOI: 10.1093/rheumatology/kez337 / PMID: 31504978

Restrictive PFT review

Date October 13, 2022

Posted by Tim Rowe

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Last week, we reviewed a differential diagnosis for restrictive lung disease with an emphasis on non-pulmonary causes. As a refresher, PAINT is a helpful mnemonic for restrictive lung disease:

Isolated mediastinal and hilar adenopathy – Review

Date October 6, 2022

Posted by Tim Rowe

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Last week in Pulmonary Report, we talked about a case of isolated mediastinal/hilar adenopathy (IMHL). 4 important differential considerations were discussed:

Infection (esp. MTB)
Sarcoidosis
Malignancy (metastatic solid organ or lymphoma)
Reactive

We discussed several studies that evaluated the optimal evaluation for suspicious IMHL, as well as factors which were shown retrospectively to be suggestive of reactive adenopathy.