Initial Sjögren’s Symptoms May Predict Severity of Lung Complications, Study Suggests

Alice Melao avatar

by Alice Melao |

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Patients with primary Sjögren’s syndrome whose first symptoms are not mouth and eye dryness may experience more progressive and severe pulmonary manifestations during the course of their disease, a study reports.

The study, “Interstitial lung disease in non-sicca onset primary Sjögren’s syndrome: a large-scale case-control study,” appeared in the International Journal of Rheumatic Diseases.

Sjögren’s syndrome is an autoimmune disease characterized by the infiltration of immune cells into secretory glands — primarily the lacrimal and salivary glands. These cells incorrectly recognize the glands’ tissue as foreign and fight it, leading to cell death and reduced production of tears and saliva, resulting in dry eyes and dry mouth, or sicca.

In some patients, however, the disease may have manifestations outside the secretory glands and cause systemic symptoms, including lung disease.

Lung symptoms appear in anywhere between 9 and 75 percent of Sjögren’s patients, depending on the diagnostic tests used.

Because systemic symptoms are the main determinants of a patient’s prognosis, early detection and treatment of such complications are a major concern among physicians.

Researchers at the Peking University People’s Hospital in China set out to compare the frequency and severity of lung disease between patients with and without sicca at disease onset.

Among the 1,341 patients admitted to the hospital between 2003 and 2012, 853 had primary Sjögren’s syndrome and 488 had secondary Sjögren’s syndrome — meaning the disease was caused by another condition.

More patients with primary Sjögren’s had lung involvement than those with secondary disease — 25.8% versus 19.3%.

Investigators then examined the clinical records of 102 primary Sjögren’s syndrome patients, hospitalized between 2008 and 2012, and whose computed tomography scans were available for analysis. The majority of these patients were women (91.2 percent), with a mean age of 61 years.

Half of them experienced sicca as their first disease manifestation. The other half had pulmonary symptoms — such as shortness of breath or coughing — joint pain, fever, blood or vessel problems, teeth loss, rashes, parotid gland swelling, or liver damage.

While the mean time from the first symptom to diagnosis was similar, more patients with sicca were diagnosed with primary Sjögren’s syndrome at disease onset than those with other symptoms — 34% versus 4%.

Non-sicca patients required salivary gland biopsies or radiological examinations of the salivary glands more often than those with sicca at disease onset. They also required more lung biopsies and lavages.

Patients without initial sicca also had lower levels of globulin, a marker of liver function and inflammation, and anti-SSA and/or anti-SSB autoantibodies, as well as elevated rheumatoid factor.

Regarding pulmonary manifestations, interstitial pneumonia was more commonly seen in non-sicca patients. Their predicted total lung capacity and forced vital capacity — measures of lung function — were also lower than patients with initial sicca symptoms.

“Non-sicca is a common initial manifestation in primary Sjögren’s syndrome with ILD,” the researchers wrote. These patients are susceptible to “more progressive and severe” pulmonary complications than sicca patients.

Comments

Ibis Longo avatar

Ibis Longo

PRE-DIAGNOSIS OF S'JOGREN'S SYNDROME

June Tousignant avatar

June Tousignant

Iam a Sjogrens patient, and started having problems breathing for 5-6 years. I have only been sent to have x-rays and breathing test. Not sure what it's called, but it's blowing into a machine.
I'm getting worse and would like to know what tests would help.
I'm in Michigan and there's a shortage of rheumatologists, and they don't really know much about Sjogrens.

Kris avatar

Kris

A relative of mine was just diagnosed with Sjogren with some iLD (interstitial lung disease) they have prescribed medicine. We’ve been getting most comprehensive information /treatment from temple lung center in Philadelphia

Shelly avatar

Shelly

I have a rheumatologist in Farmington Hills that diagnosed me with Sjögren. If that area is close, email me for more info. [email protected]

ANN PERRIGO avatar

ANN PERRIGO

My pulmonologist seems to know more about Sjogren’s than the rheumatologists did. They would just talk to me about arthritis. LOL! Kalamazoo here!

Vicky h avatar

Vicky h

I would go to Cleveland Clinic for a second opinion. They have a highly rated pulmonary and rheumatology. Please don't wait. Lung damage is irreversible and there are drugs to help lung inflammation

Shari avatar

Shari

I have sjogrens. I’m 44. It started at 35. I just couldn’t breathe. I didn’t get diagnosed until 2 yrs ago. I went from being told it’s all in my head to hypersensitivity pneumonitis to sjogrens.

There are no treatments. Inhalers, breathing treatments, tests is all you can do. I’m 1 point away from getting oxygen 88 and it needs to be 87 for oxygen.

I can’t breathe daily. The only thing that helped a little was plaquenil prescribes by rheumatologist. It has effects to your eyes irreversible they say. It suppresses your immune system.

I did a lung biopsy in the beginning and my lungs collapsed for 2 months straight. Won’t do that again. It was a
Nightmare.

Mo avatar

Mo

Hi June,
I was diagnosed about 2 to 3 months ago with Sjogren's. In November 2018 I remember having a "cold" and a lasting cough for maybe a month or so. Went to the doctor and it prompted for a X-ray, PFT. CT, blood work and bronchoscopy. I was then diagnosed with interstitial lung disease. I'm still going through testing for a solid cause of ILD. My rheumatologist wants to rule out everything else for additional treatment. I currently do not have any symptoms of Sjogren's but the least common one (lung issues). I hope this gives you some insight.
As far as meds. and treatment I am on prednisone 60mg. I was ordered oxygen for the home and I attend pulmonary rehabilitation 3x a week. I am 37 years old.

Mo avatar

Mo

I forgot to mention definitely out of breath just getting out of bed or daily activities.

MARGARITA b FRIEDMAN avatar

MARGARITA b FRIEDMAN

Doctors asked me if I had shortness of breath. I always said no. My problem was that I stopped breathing, as if my lungs got lazy. I had then to inhale consciously to start breathing again. Tha has been going for about 5 years. Now I need to do it consciously most of the time. Maybe that is shortness of breath. Does any one has this problem.?

Mo avatar

Mo

June Tousignant... I am 37 and was recently diagnosed with Sjögrens. I took chest Xrays, PFT(pulmonary function test), lung biopsy, to determine that I had interstitial lung disease cause by Sjögrens which was confirmed by a lip biopsy. I go to pulmonary rehabilitation 3 times a week which really helps along with 3 liters of oxygen. My pulmonary specialist and rheumatologist have set up a plan of medications I am set to start next week. I hooe this help given I have only been diagnosed since May 2019 with interstitial lung disease and Sjögrens since 10/2019

Teresa Quesenberry avatar

Teresa Quesenberry

What medicines are you on?

Lisa yena avatar

Lisa yena

Primary Sjogrens here was hospitalized in 2009 due to SOB. Had pneumonia with fluid up to & around my heart

Ginger avatar

Ginger

Lisa i was having chest pains on my left side. After 3 weeks went to er pain worsened. After bloodwork and ekg and ct dr said pericardial effusion. Ive had bad pneumonia a few times. Did you ever get dx with this heart condition,?

Silvi avatar

Silvi

I have been diagnosed with sjogrens for over 20 years, I am noticing lot of difficulty swallowing and in the news night time the persistent cough, very tyre, I have secondary sjogrens, RA, fibromyalgia, tyrod problems and Osteoporosis I am 56 years old so I am worry

Jessica Whorton avatar

Jessica Whorton

Spellcheck would do you some good!

Marica avatar

Marica

Jessica Wharton, I’m sorry for whatever it was that turned you into the unkind person you have become.

Jeanne Connery avatar

Jeanne Connery

How very rude.

Nonya Bis avatar

Nonya Bis

Jessica, did you enjoyed the temporary boost to your self esteem?

Personally, I found your statement pathetic, and devoid of empathy.

Margarita Friedman avatar

Margarita Friedman

How do you know if you have primary or secondary. I have recently been diagnosed with Sjogren. My doctor said I have the one that is not that dangerous. For the inflammation I have been taking Frankincense (boswellia serrata) It helps a lot. When I stop taking it, I can see the difference and how badly my arthritis has advanced. I am thinking boswellia does not cure me but only stops the swelling, and the inflammation which can cause additional damage.

Teresa Quesenberry avatar

Teresa Quesenberry

How do you take the frankincense? Do you mix it with anything? Thanks

Mo avatar

Mo

I shared this earlier but needed some outlook for people who have had this disease for a long time. I have primary Sjögrens. am 37 and was recently diagnosed with Sjögrens. I took chest Xrays, PFT(pulmonary function test), lung biopsy, to determine that I had interstitial lung disease cause by Sjögrens which was confirmed by a lip biopsy. I go to pulmonary rehabilitation 3 times a week which really helps along with 3 liters of oxygen. My pulmonary specialist and rheumatologist have set up a plan of medications I am set to start next week. I hooe this help given I have only been diagnosed since May 2019 with interstitial lung disease and Sjögrens since 10/2019

Teresa Quesenberry avatar

Teresa Quesenberry

What medications are you taking...i am having shortness of breath with multitude of other symptoms but the shortness of breath is the worse

June avatar

June

Thank you for all of your advice and your personal journeys.

It's so difficult to deal with this disease alone.
I struggle everyday with depression. Most people don't understand and assume Iam just going to get better.
Everytime I think somebody understands, they say something insensitive like what's wrong?
I had a CT to look for lymphoma and that was negative. I don't know why I can't breathe.
I am also having pain inside of my shoulder blade that gets worse when I breathe.
It would be great to see a doctor that specializes in Sjogrens. LOL
Thanks for everything.

Teresa Quesenberry avatar

Teresa Quesenberry

I am having same symptoms. I have had sjogrens for about 8 years. It started with pain in both hands...and then dry eyes...dry nose...and dry mouth...now i have extreme dry throat with shortness of breath...i have ENT appt tomorrow...hoping i find out more about severe dry throat and some swallowing difficulties. Are you on any meds for sjogrens?

Lidia Pope avatar

Lidia Pope

I am 63 years old and currently sitting in a hospital bed. I was diagnosed with sjogrens 5 years ago. Swollen hands and joints, dry eyes, dry coughing, fatigue. I was admitted to hospital for chest pains. Have had them a month. Just had all heart labs/echos/stress test/nuclear dye injected, etc. Things coming back negative. But chest pain won't go away. It's worrisome. Chest x-rays were clear. Anyone also experiencing chest pain/tightness/pressure?

Denise avatar

Denise

Yes, I was hospitalized for the same thing and thought I was having a heart attack. Turns out, I have GERD and a sliding hiatus.

Dianna Schmidtberger avatar

Dianna Schmidtberger

I am 57 and have recently been diagnosed with Sjogrens. However I am not new to the horrible problems this diease can cause. My mother passed away at 62 from Sjogrens at the time they didn't know much about it and by the time they found the plaque in her spine it was to the brain. I am having many issues and have started the road of testing. Lip biopsy, lung test and see a neurologist for and MRI. Reading your replies on the lung issues is definitely eye opening and scary as I'm having this issue and a cough for many years now that I was told was allergies. Wishing you all the best and we are all in this same boat together ❤️

Jeanne Connery avatar

Jeanne Connery

How very rude.

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