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

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

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.

19 comments

  1. June Tousignant says:

    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 says:

      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

    • ANN PERRIGO says:

      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 says:

      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 says:

      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 says:

      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.

    • MARGARITA b FRIEDMAN says:

      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 says:

      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

  2. Lisa yena says:

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

    • Ginger says:

      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,?

  3. Silvi says:

    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

  4. Nonya Bis says:

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

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

    • Margarita Friedman says:

      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.

  5. Mo says:

    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

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