respiratory
Research

TSANZ: call for sarcoidosis research


A State of the Art Paper on Sarcoidosis has highlighted the need for more advocacy and research around the aetiology, prevention and management of the disease.

The TSANZ resource, also summarised in the MJA, is the first update on sarcoidosis for over a decade and the first written in Australia.

Lead author Professor Paul Thomas told the limbic that some advances, such as whole body PET scans to identify local disease activity, were difficult to access as they were not reimbursed for sarcoidosis.

However endobronchial ultrasound guided fine needle aspiration of lymph nodes was working quite well for diagnosing people with sarcoidosis.

“In terms of monitoring people with sarcoidosis we don’t have any specific new measures, and it would be good to get some more innovative way of looking at the inflammation, particularly in the lungs from our perspective, but also any other markers of systemic sarcoidosis would be good.”

Professor Thomas, from the department of respiratory medicine at the Prince of Wales Hospital in Sydney, said it was important to have biomarkers that could distinguish active disease from more indolent disease as many patients would not need any treatment.

“It depends how florid the disease is. If it presents acutely, they might require treatment but if the disease appears to be either indolent or has come on acutely and is regressing, then you wouldn’t want to add treatment especially steroids and drugs like methotrexate and mycophenolate which have significant side effects. You don’t want to be treating something which is going away already.”

Professor Thomas many patients went undiagnosed or presented with non-specific symptoms such as chronic tiredness. Other patients presented with eye symptoms, a dry cough or unexplained lymphadenopathy,

Elevated serum angiotensin-converting enzyme (ACE) might a clue to further investigations but had modest sensitivity and specificity for diagnosis.

Pulmonary function tests were abnormal in about 20% of patients with mild disease and up to 70% with more advanced disease. Airway hyper-responsiveness was common but unlike in asthma, there may not be a response to bronchodilators.

Professor Thomas said the heterogeneity of the disease was a challenge.

“Different specialists focus on different problems and it is always important for us as respiratory physicians to get people’s eyes checked and to make sure they have some baseline monitoring of their cardiac function because just occasionally you can run into trouble with sarcoidosis affecting the eyes and the heart.”

He said dysrhythmias could happen but were rare in Australia.

The MJA article said TNF inhibitors such as infliximab and adalimumab, typically used after steroids, methotrexate and other oral immunosuppressants had failed, had modest effects.

“Other agents such as anti-CD20 (rituximab) have yet to be studied in a controlled manner, but logically, an intervention that targets CD4 cells or the Th1 (and perhaps Th17) pathways would be of potential benefit. Problems include cost and availability.”

It called for better collaborative networks and a multidisciplinary approach to advance clinical research and trials. Professor Thomas said a national registry would also be welcome.