By: Dr. Hassan Baig, MD, M.B.B.S.
Assistant Professor in Medicine
Pulmonary and Critical Care Medicine
Director, ILD Clinic Mayo Clinic Florida
Navigating any chronic illness can be challenging but understanding the various aspects of diagnosis and management can help ease the journey. Sjögren's disease (SjD) can present in a variety of ways as it can affect different body systems. The pulmonary system manifestations are several and can have overlapping symptoms including shortness of breath or cough. With a thorough evaluation, a better understanding of the extent of involvement can be achieved which will allow for the appropriate management.
Pulmonary Involvement in Sjögren’s disease
Long standing cough is a common symptom and is usually due to the dryness involving the airways including the trachea. Interstitial lung disease (ILD) is a process that results in inflammation and scarring in the lung tissue, which can affect 10-20% of patients with SjD. Other manifestations include lung nodules, air cysts, and inflammation of the small and large airways. Most of these conditions can present with cough or shortness of breath, but sometimes patients remain asymptomatic.
Diagnosis of ILD
If symptoms are present, assessing the lung function and structure should be the next steps. Complete pulmonary function testing (PFT) measures a person’s ability to move air in and out of the lungs, the total lung capacity and oxygenation ability. It is important to note that sometimes even if disease is present, the PFT may be normal. In those cases especially, a computed tomography (CT) of the chest will be helpful. The CT scan provides a detailed look at the lungs and can identify most problems including ILD. In some cases, a lung biopsy may be considered if the diagnosis remains unclear.
Types of ILD
When inflammation or scarring is noted on CT chest imaging, certain patterns of lung injury can be identified. Terms like non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia (UIP), or lymphocytic interstitial pneumonia (LIP) were originally used by pathologists when examining lung biopsy specimens. Each category is a description of the pattern of lung tissue injury with varying degrees of inflammation, scarring and/or cyst formation. NSIP, the most common type ILD type in SjD, typically involves more of an inflammatory component which may be a target for treatment. UIP is primarily a fibrotic or scarring process, while LIP is characterized by dense inflammation to a greater extent than NSIP. These biopsy patterns have been correlated with specific imaging patterns seen on CT chest scans, so when a term like NSIP is found on the CT imaging report, it means that the underlying lung pathology (if biopsy was done) should also be NSIP. With the availability of high-quality CT scans, the need for lung biopsy to identify the ILD type has become less common. It is important to define the type of ILD as it can affect treatment and prognosis.
ILD Treatment
The need for treatment depends on the extent of disease burden determined by symptoms, pulmonary function impairment, and degree of inflammation or scarring on CT imaging. If the disease is noted to be mild, then follow up with serial testing may be appropriate. If moderate to severe disease is present, then treatment with immunosuppressive medications is usually recommended. Sometimes anti-fibrotic medications are added if there is increased scarring. Routine follow with serial pulmonary function testing is recommended to monitor for stability or progression. Some patients may not have lung involvement at the time of SjD diagnosis. If they develop persistent cough or shortness of breath, then it is recommended to pursue evaluation with a pulmonologist.
By: Dr. Hassan Baig, MD, M.B.B.S.
Assistant Professor in Medicine
Pulmonary and Critical Care Medicine
Director, ILD Clinic Mayo Clinic Florida
Navigating any chronic illness can be challenging but understanding the various aspects of diagnosis and management can help ease the journey. Sjögren's disease (SjD) can present in a variety of ways as it can affect different body systems. The pulmonary system manifestations are several and can have overlapping symptoms including shortness of breath or cough. With a thorough evaluation, a better understanding of the extent of involvement can be achieved which will allow for the appropriate management.
Pulmonary Involvement in Sjögren’s disease
Long standing cough is a common symptom and is usually due to the dryness involving the airways including the trachea. Interstitial lung disease (ILD) is a process that results in inflammation and scarring in the lung tissue, which can affect 10-20% of patients with SjD. Other manifestations include lung nodules, air cysts, and inflammation of the small and large airways. Most of these conditions can present with cough or shortness of breath, but sometimes patients remain asymptomatic.
Diagnosis of ILD
If symptoms are present, assessing the lung function and structure should be the next steps. Complete pulmonary function testing (PFT) measures a person’s ability to move air in and out of the lungs, the total lung capacity and oxygenation ability. It is important to note that sometimes even if disease is present, the PFT may be normal. In those cases especially, a computed tomography (CT) of the chest will be helpful. The CT scan provides a detailed look at the lungs and can identify most problems including ILD. In some cases, a lung biopsy may be considered if the diagnosis remains unclear.
Types of ILD
When inflammation or scarring is noted on CT chest imaging, certain patterns of lung injury can be identified. Terms like non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia (UIP), or lymphocytic interstitial pneumonia (LIP) were originally used by pathologists when examining lung biopsy specimens. Each category is a description of the pattern of lung tissue injury with varying degrees of inflammation, scarring and/or cyst formation. NSIP, the most common type ILD type in SjD, typically involves more of an inflammatory component which may be a target for treatment. UIP is primarily a fibrotic or scarring process, while LIP is characterized by dense inflammation to a greater extent than NSIP. These biopsy patterns have been correlated with specific imaging patterns seen on CT chest scans, so when a term like NSIP is found on the CT imaging report, it means that the underlying lung pathology (if biopsy was done) should also be NSIP. With the availability of high-quality CT scans, the need for lung biopsy to identify the ILD type has become less common. It is important to define the type of ILD as it can affect treatment and prognosis.
ILD Treatment
The need for treatment depends on the extent of disease burden determined by symptoms, pulmonary function impairment, and degree of inflammation or scarring on CT imaging. If the disease is noted to be mild, then follow up with serial testing may be appropriate. If moderate to severe disease is present, then treatment with immunosuppressive medications is usually recommended. Sometimes anti-fibrotic medications are added if there is increased scarring. Routine follow with serial pulmonary function testing is recommended to monitor for stability or progression. Some patients may not have lung involvement at the time of SjD diagnosis. If they develop persistent cough or shortness of breath, then it is recommended to pursue evaluation with a pulmonologist.
Does your Doctor have the Sjögren's Foundation's Pulmonary Clinical Practice Guidelines?