THE ACHES THAT TAKE YOUR BREATH (AND TEARS) AWAY

Area of Science:

• Rheumatology
• Pulmonology
• Immunology

Background:

• Sjögren's syndrome (SjS) is a chronic autoimmune disease primarily affecting exocrine glands, but can manifest with systemic complications.
• Interstitial pneumonitis is a serious pulmonary manifestation of SjS, impacting lung function and patient prognosis.
• Management of SjS-related lung disease often requires a multi-faceted approach, including immunosuppressants and targeted therapies.

Purpose of the Study:

• To describe the clinical presentation and management of an elderly male patient with Sjögren's syndrome presenting with interstitial pneumonitis.
• To evaluate the efficacy of immunosuppressive therapy, including rituximab, in managing pulmonary function and systemic symptoms in SjS.
• To illustrate the challenges in managing chronic autoimmune conditions with significant organ involvement.

Main Methods:

• Case report of an 80-year-old male with Sjögren's syndrome, fatigue, arthralgias, and respiratory symptoms.
• Diagnostic workup included physical examination, laboratory tests (Anti-Ro/SSA, Anti-La/SSB, ESR, CRP), pulmonary function tests (FVC, DLCO), and high-resolution chest CT.
• Treatment involved initial immunosuppression (mycophenolate mofetil, hydroxychloroquine, prednisone) followed by rituximab for refractory symptoms and pulmonary decline.

Main Results:

• The patient presented with classic SjS symptoms and evidence of nonspecific interstitial pneumonitis with significantly reduced FVC (64%) and DLCO (44%).
• Initial treatment led to symptom improvement and a 20% increase in FVC, but tapering prednisone caused worsening dyspnea.
• Subsequent rituximab administration resulted in overall symptom improvement and a sustained increase in FVC to 71%, despite intermittent fluctuations.

Conclusions:

• Sjögren's syndrome can present with significant interstitial lung disease, requiring aggressive management.
• Rituximab appears to be a viable therapeutic option for managing refractory pulmonary involvement in SjS, improving lung function and symptoms.
• Long-term management of SjS-related interstitial pneumonitis may necessitate repeated courses of biologic therapy to maintain clinical stability.