Lung Complications and Autoimmune Disease from the Sjogren's Conference
I've finally gotten another report typed up! This one should be helpful for those who are having lung and breathing issues.
Lung Complications and Sjogren’s Richard T. Meehan, MD, FACP, FACR
Dr. Meehan is from the National Jewish Health Hospital in Denver, Colorado. It is very impressive that he considers it important that patients who present with pulmonary symptoms should also be checked for an underlying AI disease. He said that sometimes the lung can provide the first clue in AI disease.
Clinical History which should point to an AI disease investigation:
• Typical sicca symptoms
• Parotid or lymph gland enlargements
• Refractory sinusitis – constant sinus disease
• Increased dental caries
• Bronchiectasis and unusual infections
• Interstitial Lung Disease manifested by exertional breathlessness and non-productive cough
• Pleurisy, unintentional weight loss, fevers, fatigue, joint and muscle pain
• Impaired exercise capacity
Laboratory evaluations are then ordered to determine if there is any systemic inflammation.
Besides the usual laboratory blood work for AI diseases, Dr. Meehan outlined the pulmonary manifestations of SJS.
• Diffuse Lung Disease/ ILD (Interstitial lung disease)
• Airspace Consolidation/ Ground Glass Opacification
• Airway Damage
• Serositis (pleural or pericardial thickening) This can be seen in underlying lupus.
NJH Clinical experience - Over the course of 18 months 244 patients were diagnosed with primary or secondary SJS. Many were referred for ILD or bronchiolitis with no prior suspicion of SJS.
Therapeutic Approach and Management
Immune suppressive drug choice:
• AZA (Imuran) - for milder or slowly progressive disease
• CYC (Cytoxan)- probably most effective against ILD, but risky
• MMF (Cellcept)- less toxic than CYC, may be better tolerated than AZA.
• Cyclosporine - may be effective in patients with intolerance to above.
• Tacrolimus - Similar to cyclosporine, more expensive but may be better tolerated.
• Combinations - Infliximab, Cellcept or AZA helpful in RA associated Bronchiolitis.
• Rituximab - may be helpful in LIP.
• Mild or non progressive bronchiolitis may require only Hydroxychloroquine (Plaquenil) and “watchful waiting”.
The ideal immunomodulator or immune suppressive therapy for SJS patients with ILD has not been identified, and Dr. Meehan is calling for multicenter SJS treatment trials.