THE ACHES THAT TAKE YOUR BREATH (AND TEARS) AWAY.

THE ACHES THAT TAKE YOUR BREATH (AND TEARS) AWAY.

J La State Med Soc. 2015 May-Jun;167(3):154-155

Authors: Becerril Md J, Gonzales Md H, Saketkoo Md LA

Abstract
CASE: An 80-year-old man presented with a complaint of three months of fatigue and aching of his shoulders and hips, as well as pain, swelling, and stiffness in bilateral fingers that was worse in the morning but improved with movement. Associated symptoms included worsening dry mouth and eyes, dysphagia, exertional dyspnea, and right foot drop. Physical exam was significant for edematous and tender bilateral proximal interphalangeal joints, metacarpophalangeal joints and wrists with decreased grip, extension and flexion, as well as bilateral pulmonary crackles. Laboratory analysis revealed Anti-Ro (SSA) and Anti-La (SSB) positivity with elevated erythrocyte sedimentation rate (70mm/hr) and C-reactive peptide (13mg/L). Pulmonary function testing was notable for a forced vital capacity (FVC) of 64% and carbon monoxide diffusing capacity (DLCO) of 44%. High resolution chest computed tomography demonstrated fibrotic changes consistent with nonspecific interstitial pneumonitis. The patient was started on mycophenolate mofetil, hydroxychloroquine, and prednisone for Sjögren's syndrome (SjS). Symptoms improved and repeat FVC revealed a 20 percent improvement, however subsequent tapering of prednisone resulted in worsening dyspnea and increase of FVC to 60 prcent. Prednisone was restarted and rituximab 2g divided in two doses was administered with overall symptom improvement. Symptoms and FVC continued to wax and wane over the following 18 months requiring re-dosing of rituximab with most recent FVC improved to 71 percent and DLCO 41 percent.
DISCUSSION: SjS is a connective tissue disease (CTD) of unknown etiology characterized by lymphocytic infiltration of several organ systems. Though the quintessential presentation is with decreased salivary and lacrimal gland function, common manifestations of SjS include arthritis, neuropathy, and interstitial lung disease (ILD). While reported rates of lung involvement in SjS vary widely (9-75%) the development of ILD in SjS has been associated with worse survival. ILD can be difficult to manage in patients with CTD such as SjS, as it is often progressive with a paucity of data on therapeutic options. ILD in CTDs such as SjS carry a high burden of morbidity, however early recognition and appropriate treatment, including novel therapies such as rituximab, may impact survival and reduce symptoms.

PMID: 27138413 [PubMed - as supplied by publisher]

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