The connective tissue diseases, also called collagen vascular diseases (CV), are a group of multisystem diseases. They include, but are not limited to, systemic lupus erythematosus (SLE), Sjogren’s syndrome (SS), systemic sclerosis (SSc), rheumatoid arthritis (RA), Raynaud’s phenomenon, and the vasculitides. These are autoimmune diseases characterized by pathologic changes in the blood vessels and connective tissues. SLE is the second most common of the connective tissue diseases. It is a multisystem disease with a wide variety of clinical manifestations, ranging from a benign, easily treated disease with a rash and arthritis to a life-threatening illness with progressive nephritis or central nervous system damage. The histopathologic hallmark of SS is focal lymphocytic infiltration of the salivary glands, lacrimal glands, or extraglandular organs without structural destruction. The biopsy is usually taken from a minor salivary gland on the inside of the lower lip by an oral surgeon.