Abstract
Sjögren's syndrome (SS) is one of the most common autoimmune disorders. The disorder primarily affects the exocrine glands of the eyes and mouth and is regularly associated with extraglandular manifestations. These extraglandular manifestations can range from pulmonary, cardiac, muscular, neurological, to vascular. As the disease progresses, a majority of SS patients eventually develop extraglandular manifestations. Approximately 20% of patients with SS will develop pulmonary manifestations. However, it is uncommon to have the presenting symptoms be extraglandular and even less common to have multiple organs involved or critical illness. We present the case of a 42-year-old female whose only presenting symptoms were extraglandular, affecting multiple organs and leading to critical illness.