Wednesday, May 14, 2014

Sjogren's Syndrome Foundation: Resources for Patients With Sjogren's Related Pulmonary Issues

I'm currently being a very good post-ESI (epidural steroid injection) patient by chilling my bum regularly. Well, more accurately I'm applying ice at intervals to my L5 and S1 areas of my spine.

So while I'm cooling my injection site to help encourage the injected steroid to hang around the area of my spinal stenosis as long as possible, I've been doing a bit more reading. I was intrigued by a little blurb in the latest issue of Sjogren's Quarterly, a periodical published by the SSF for health care providers:

A new pulmonary hypertension drug, Opsumit®, has received the green light from the U.S. Food and Drug Administration (FDA). The development of Actelion’s new drug follows the company’s loss of patent protection in 2015 for another pulmonary hypertension therapy, Tracleer®. Ospumit® is an endothelin receptor antagonist which relaxes pulmonary arteries and decreases arterial pressure. Although uncommon, pulmonary hypertension has been noted to occur in relation to Sjögren’s. 
For treatment of all pulmonary complications in Sjögren’s, see the list of Pulmonary Clinics treating Sjögren’s patients on the Sjögren’s Syndrome Foundation website found under both “Treatments” and “Provider/Researcher” sections at
I've included the list of Pulmonary Clinics treating Sjogren's patients from 2013 here:

March 25, 2013

Sjögren’s is the second most prevalent autoimmune rheumatic disease and affects about 4 million Americans. In addition to affecting the moisture producing glands resulting in hallmark symptoms of dry eye, dry mouth, fatigue and joint pain, Sjögren’s can affect any body organ or system. Interstitial lung disease is the most common pulmonary manifestation in Sjögren’s, but pulmonary hypertension, amyloidosis, cystic lung disease and MALT lymphoma can also occur in the lungs.

Few studies have been done in pulmonary manifestations of Sjögren’s, and few pulmonary experts who are also knowledgeable about Sjögren’s have been available to Sjögren’s patients. The Sjögren’s Syndrome Foundation applauds the vision to create Sjögren’s Pulmonary Clinics within the LAM clinical settings to change this. Our hope is that these clinics will provide our patients with expert specialized care and lead to an expanded interest in pulmonary manifestations in Sjögren’s and future studies that will increase our knowledge and improve future care in this field.

The Sjögren’s Syndrome Foundation is pleased to partner with the LAM clinics in this important endeavor.

26 Pulmonary Clinics included in the LAM Network that will be expanded to become Sjogren’s Pulmonary Clinics:

University of Alabama at Birmingham
Birmingham, AL
Joseph Barney, MD FACP, FCCP

Mayo Clinic-Scottsdale
Scottsdale, AZ
Richard Helmers, MD
Laszlo Vaszar, MD

ULCA Clinic- Los Angeles
Los Angeles, CA
Joseph P. Lynch III, MD

University of California
La Jolla, CA
Gordon Yung, MD

University of California, San Francisco
San Francisco, CA
Joyce Lee, MD

Stanford University Medical Center
Stanford, CA
Glenn Rosen, MD

National Jewish Health
Denver, CO
Kevin Brown, MD
Greg Downey, MD

Mayo Clinic- Jacksonville
Jacksonville, FL
Charles Burger, MD
Augustine Lee, MD

University of Miami
Miami, FL
Marilyn Glassberg

Emory University School of Medicine
Atlanta, GA
Gerald Staton, MD
Sirhari Veeraraghavan, MD, FCC

Loyola University Medical Center
Maywood, IL
Dan Diling, MD
Emily Gilbert, MD

Brigham and Women’s Hospital
Boston, MA
Elizabeth Henske, MD
Souheil El-Chemaly, MD

University of Michigan
Ann Arbor, MI
Kevin Flaherty, MD, MS
MeiLan Han, MD, MS

Mayo Clinic- Rochester
Rochester, MN
Eric Olson, MD
Jay Ryu, MD
Misbah Baqir, MBBS

Washington University School of Medicine/Barnes Jewish
St. Louis, MO
Mario Castro, MD, MPH
Adrian Shifren, MD

New York
New York, NY
Jeanine D'Armiento, MD, PhD

University of Rochester Medical Center
Rochester, NY
Patricia Sime, MD, FRCP

University of Cincinnati Medical Center
Cincinnati, OH
Frank McCormack, MD

Cleveland Clinic
Cleveland, OH
Joseph Parambil, MD

Oregon Health and Science University
Portland, OR
Alan Barker, MD

University of Pennsylvania
Philadelphia, PA
Robert Kotloff, MD
Maryl Kreider, MD

South Carolina
Medical University of South Carolina
Charleston, SC
Charlie Strange, MD

Vanderbilt University Medical Center
Nashville TN
Lisa Young, MD

University of Texas Southwestern Medical Center
Dallas, TX
John Fitzgerald, MD
Carlos Girod, MD

UT Health Pulmonary Clinic
Houston, TX
Brandy McKelvy, MD
Rima Gidwani, MD

Swedish Medical Center
Seattle, WA
George Pappas, MD

Also included this issue was the most recent and excellent patient information sheet which has yet to be listed on the SSF patient information website:

Patient Education Sheet 
Sjögren’s-Related Lung Disease
The SSF thanks Teng Moua, MD for authoring this Patient Education Sheet. Dr. Moua is affiliated with the Mayo Clinic, Division of Pulmonary/Critical Care Medicine, Rochester, Minnesota.

One feature defining Sjögren’s is the inappropriate infiltration of certain white blood cells into glandular tissues known as ‘exocrine’ glands. This infiltration contributes to reduced tear and saliva production, causing the classic symptoms of dry eyes and dry mouth associated with Sjögren’s. Because the airway and lungs are lined with exocrine glands, the lung is commonly involved in Sjögren’s, with respiratory symptoms occurring in up to two thirds or more of patients. Some fast facts:

  •  Parts of the lung that may be involved in Sjögren’s include the upper and lower airways, the small or microscopic airways, and the lung tissue or air sacs and their supportive structures.
  •  The most common respiratory symptom is a dry cough or airway irritation referred to as ‘xerotrachea’ or ‘dry airway’.
  •  Other symptoms include cough productive of sputum, wheezing, and shortness of breath both at rest and with exertion.
  •  Doctors may perform breathing tests (pulmonary function tests (PFT)) to assess for abnormalities and order chest x-ray and special radiologic testing such as computed tomography (CT) to help characterize the extent of lung involvement.
  •  Findings on radiology may include patchy infiltrates or locally consolidated lung suggesting inflammation or infection. Cysts or small thin-walled air pockets in the lung and thickened or elongated airways are other findings seen in Sjögren’s.
  • Other dryness symptoms and blood inflammatory markers for Sjögren’s often do not correlate with the likeli- hood or severity of respiratory symptoms.
  • Occasionally, Sjögren’s may be associated with another autoimmune or inflammatory disease that can affect the lung. In this case, lung disease may be worse in terms of severity and progress more rapidly over time.
  • Many medications used to treat Sjögren’s may cause unintended side effects that injure the lung. A careful review is warranted, particularly if symptoms develop after starting a new medication.
  • Medications that suppress the immune system may also increase the likelihood of lung infection. A careful assessment for possible infection is often the first step in managing new respiratory symptoms.
  • Finally, if infection and medication side effects are ruled out, therapy directed at treating the underlying Sjögren’s may need to be adjusted in an attempt to treat respiratory symptoms.
  • Supportive treatments such as inhalers or oxygen in severe disease can be helpful for managing symptoms. Pulmonary clinics with a special interest in Sjögren’s are listed on the SSF website.

For more information on Sjögren’s, contact the Sjögren’s Syndrome Foundation at:
6707 Democracy Blvd, Suite 325, Bethesda, MD 20817 • 800-475-6473 • •

Clinicians: Please make multiple copies of this Patient Education Sheet and distribute to your patients.


Heda said...

I wish I could get to one of those clinics. My cough is my worst symptom. I cough up bucket loads of phlegm literally. Typhoid Annie has nothing on me. People move away from me on public transport. I no longer go to the movies. Any gathering with lots of people is out. Conferences, church, trains, planes, etc. Restaurants are tricky and sometimes work and sometimes don't. One of my cysts burst and I had a spontaneous pneumothorax. I'm so tired of lung problems. There is a recent finding that with Sjogren's it's possible to have a cough that is generated by neuropathy. Rings true to me. Thank you so much for your post. Can't tell you how grateful I am.

Dotti Bee said...

Heda, Get your sputum tested (what you cough up!) Mine showed a bacteria that is common in COPD. I actually got much better after a month on an antibiotic to avoid malaria while on a trip to India, but back to dry cough after the winter bug. The Gp's and even a repiratory specialist seem to not understand these probs and don't want to give out meds(would avoid if i could). I'm ready to retire too. live near Sydney. cheers Dot

Dotti Bee said...

Yes, just bought 5 books on Alzheimers and started doing Luminosity. Memory is my worst element. Sjogrens for 10 years, probably for 25. Now 62- Raynauds and dry dry eyes. Lungs going downhill. Menopause before diagnosis, so that was long and icky and a little sweaty. Will try to get through all of blog. Books just have 'same ole, same ole'. ;0)