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    Ask the Expert: Dr. Nishant Gupta

    Nishant Gupta, MD

    University of Cincinnati

    Board Member, Sjögren’s Foundation

Interstitial Lung Disease (ILD) and Prevalence in Sjögren’s

Interstitial lung disease (ILD) refers to a broad group of disorders that can cause inflammation and/or scarring in the lungs. Over time, this can progress to cause symptoms such as shortness of breath and chronic cough, pose limitations in daily life, and in severe cases be life threatening. Many different insults such as environmental exposures, viral infections etc. can lead to the development of ILD, including autoimmune diseases such as Sjögren’s disease. In fact, ILD can be seen in about 10-15% of patients with Sjögren’s.

ILD Symptoms in Sjögren’s

The most common symptoms from ILD include shortness of breath and cough; however, early in the disease course patients may be relatively asymptomatic. This is one of the reasons behind the recommendation to actively screen for lung involvement in patients with Sjögren’s in the recently published  Guidelines for Pulmonary involvement in Sjögren’s. It is also worth noting that ILD may be the presenting manifestation of Sjögren’s disease. Thus, it is important to ask about symptoms that might clue towards the possibility of underlying Sjögren’s disease (or other autoimmune diseases) when evaluating patients with ILD.

Diagnosis of ILD

The best non-invasive way to look for ILD is via chest CT scan. Chest CT can not only help answer whether there is ILD but can also provide useful information regarding the type and severity of ILD. Patients with Sjögren’s, especially those with pulmonary symptoms such as shortness of breath and/or cough that lasts beyond a few weeks, should have a low threshold to obtain chest imaging. Lung biopsy is generally not needed in patients with underlying Sjögren’s; however, may be required in some instances on a case-by-case basis depending upon the underlying features and individual circumstances.

Complications of ILD in Sjögren’s Patients

In addition to the inflammation and scarring typically seen in all ILDs, patients with Sjögren’s tend to develop a unique form of lung involvement where air gets trapped within the lungs and causes the formation of multiple air-filled pockets, known as cysts. These cysts might make patients with Sjögren’s more likely to experience sudden onset lung collapse, also known as pneumothorax. As such, it is important for patients with Sjögren’s to be aware of the most common symptoms of a pneumothorax - sudden onset shortness of breath and chest pain that is worsened by deep breathing. The development of these symptoms should prompt patients with Sjögren’s to seek immediate medical attention.

Treatment of ILD

Patients with ILD require long-term monitoring to better understand the disease trajectory and to help make treatment decisions. This goal is typically accomplished by performing serial pulmonary function tests (PFTs). Patients with minimal symptoms and normal to mildly affected PFTs, especially those with stable PFTs over time, may not require any specific treatment beyond supportive care. On the other hand, patients with significant symptoms and/or moderate-severe derangement in PFTs or progressive disease often require treatment for their ILD. General modalities for treatment include immunosuppressive therapies with consideration of antifibrotics in patients who progress despite adequate immune suppression. Detailed description and suggested algorithms for the management of ILD in patients with Sjögren’s is available here.

If you have any further questions about your medical condition, please seek advice from your physician.


— Sep 8, 2023

just had a
TAVR procedure and still having shortness of breath in extremely cold or hot weather Could this be the Sjogrens? do they have any newer medications for sjogrens fatigue? I suffer so bad with fatigue and soreness throughout my body

Hi Isabella, just found this website and saw your question. I think dietary changes could really help reduce your symptoms. I found having vegetable smoothies (see Goodbye Luous website) with flaxseed (for Omega 3s) really helped me and i could go off Plaquenil.

— Sep 21, 2023

I have both Sjogrens and ILD and I'm due to have a cancer lesion on my face removed and plastic surgery repair.
Is it safe to have a general anaesthetic as my month is consistently servilely dry?
Thank you

— Sep 23, 2023

Thanks for the informative article. It made me feel a bit better. I've had Sjogrens for 20+ years and only recently been diagnosed with ILD from a CT done for something unrelated. I have no symptoms and have slightly lower diffusion based on PFT but I can still hike/bike at high altitude (9000+ft). I wondered if doctors were doing enough for my ILD but your article made it sound like they are making the right recommendations for now.

— Nov 23, 2023

Recently diagnosed with Lymphocytic Interstitial Pneumonia. New CT scan shows air filled cysts and atelectasis. Waiting to hear from pulmonologist.

— Feb 22, 2024

I have rheumatoid arthritis and Sjogrens Syndrome with ILD first diagnosed in 2018 - multiple cysts which currently don’t cause any problems whatsoever. I’m waiting to have a complete shoulder replacement and am hoping that given the small chance of pneumothorax I will not be considered at risk when having a general anaesthetic?

Hi Jackie, I just discovered this site for the first time today. I have been trying to figure out what is going on with me especially beginning to think all the various ailments and conditions I have had throughout the years are somehow related. I stopped to comment when I read that you are waiting for a complete shoulder replacement. I have postponed having Rotator Cuff Surgery called Reverse Shoulder Replacement on both my shoulders since 2021 Sept. timeframe. My Pulmonologist is also checking to see what kind of Lung Disease I have and it is all so confusing especially when I think some Dr's, as great as they are, seem to not really associate all my past issues, which I have indicated don't seem to be possibility related. I apologize if this is not something similar to what you are going through. I am very tired and hope I make sense to you. Thank you for your time.

Hi Donna, I apologise for the late reply - I’ve only just seen your post. What you’re going through sounds very similar to me. I have now been told that I’m something of a poor risk anaesthetic wise due to LIP (lymphatic interstitial pneumonia) pattern ILD (interstitial lung disease) which could lead to a pneumothorax - collapsed lung - whilst under anaesthetic. I have discovered that what I previously thought were cysts are actually holes in my lungs. They don’t currently cause any problems and haven’t got any worse in the last 4 years (evidence on MRIs).
Reverse shoulder replacement has been postponed for now, instead I’m doing specialist physio “the Torbay protocol for massive rotator cuff tears” ! - you can find it online, the exercises are painful to do but are helping with range of movement. I’m seeing the surgeon again in 3 months time for a review. To add insult to injury my other shoulder is going the same way which is making everyday life very difficult and frustrating.
It seems to be very difficult to find a rheumatologist that is informed on all the complications of Sjogrens Syndrome - I feel your pain ! I’d be interested to hear if you are making any progress.

I've had Sjogrens for 20+ years and was diagnosed with ILD- pulmonary fibrosis in August of 2020. I'm mostly managed on prednisone and CellCept. I had a shoulder replacement done in the Fall of 2021. I had a consult with the hospital's anesthesia department after scheduling. It really helped with the decision. I did well and didn't have any issues post op. Of course this is just my experience.

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