RF UK MAP London Nottingham

A newly established network for rare cystic lung diseases

Wednesday, March 31, 2021

NHS England has designated the Royal Brompton Hospital in London as the lead centre for a new national rare disease collaborative network which focuses on cystic lung disease.

The network was developed in collaboration with, and includes the Nottingham University Hospitals NHS Trust, itself an institution known for its expertise in the management of patients with cystic lung diseases and already commissioned by NHS England for the management of patients with lymphangioleiomyomatosis (LAM).

We asked the engineers of the establishment of the network, Dr Peter George, Consultant at the Royal Brompton Hospital London and lead for the interstitial lung disease service, and Professor Simon Johnson, Head of the Division of Respiratory Medicine at the University of Nottingham, what the network brings to people affected by rare cystic lung diseases.

Dr George, could you tell us which conditions the network is supporting?

Cystic lung diseases are a heterogenous group of rare conditions such as lymphangioleiomyomatosis (LAM), Langerhan’s Cell Histiocytosis (LCH), Lymphocytic Interstitial Pneumonia (LIP), Birt-Hogg-Dubé syndrome (BHD), and others.

Although these conditions may all appear very similar on CT, there are important differences which affect the outlook for affected patients, the type of monitoring that is required, the organs outside the lungs that are involved and the therapeutic options. Without specialist input, patients may remain misdiagnosed for years meaning that their disease can progress and symptoms can worsen. Additionally as the diseases have variable rates of progression, a definitive diagnosis and individual assessment are required to determine the optimal treatment and needs of each patient.

How did the idea of forming a network come together?

This initiative was led by myself and Professor Simon Johnson, who is the Lead for the National Centre for LAM in Nottingham.

Both our institutions benefit from decades of world-leading expertise in the management of patients with cystic lung disorders, have an excellent longstanding mutual working relationship and both have well-established referral links for these conditions.

The Royal Brompton Hospital has a dedicated weekly cystic lung disease clinic and a monthly specialist Multi-Disciplinary Team meeting (MDT) led by Dr Maria Kokosi, and a specialized LCH service, while Nottingham University Hospitals NHS Trust hosts the NHS England Commissioned National Centre for LAM, and will continue to sub-specialize in this condition even as part of the network.

It made sense for both to join in a more formal collaboration, which allowed us to put forward a comprehensive proposal to develop and improve the care of patients with rare cystic lung diseases throughout the UK. The network will improve access to novel therapies, highly specialized multidisciplinary teams and clinical trials. Furthermore, through our joint MDTs, we are able to share expertise and discuss the most complex of these cases drawing on the benefits of our clinical, radiology and pathology teams.

What expertise and services can you offer to patients, now that you are a network?

As part of a network, our institutions can provide their patients with specific treatments such as mTOR inhibitors (sirolimus), chemotherapy, and immunomodulation, and our diagnostic capabilities have increased too. We can now offer advanced imaging modalities, dedicated genetics services, on site thoracic surgery, cryobiopsy, BAL immunohistochemistry and biomarker testing such as serum VEGF-D.

Having access to each-other’s pool of expertise means that patients will benefit from multi-specialty assessments which include dermatology, urology, oncology, haematology, pulmonary hypertension, but also transplant teams and thoracic surgery.

Another feature is a quarterly network-wide Multi Disciplinary Team meeting, which brings together a body of expert Radiologists, Pathologists, and ILD specialists to discuss cases referred to the network. We are fortunate to work with the some of the best and most experienced radiologists and pathologists in the world and they are a hugely important part of our team and the service.

One of the goals of our network is to ensure access to our teams regardless of geographic location. By having one centre in London and another in Nottingham we partly address this but in future plan to expand the network by inviting additional specialist centres from across the UK.

What are the plans for the network?

The rare disease network is first and foremost there to provide the highest quality care for patients with cystic lung diseases and to standardise the quality of care for these rare conditions, encouraging the sharing of expertise and best practice.

But we want it to go beyond diagnosis and treatment. We want this collaboration to facilitate research into the understanding of these diseases, so we also aim to create a national cystic lung disease registry.

This will provide accurate prospective data with regards the incidence and prevalence of the different cystic lung diseases, and facilitate research on the pathogenesis, clinical characteristics and treatment of these conditions.
In the future we also hope to develop guideline statements for the management of rare cystic lung diseases.