In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1933, Henrik<unk>  was the first to describe 19 females with clinical and pathological manifestations of the<unk> . American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for<unk>  in 2002 and 2012 according to clinical findings. Common causes of<unk>  include viral infection such as Epstein-Barr virus (EBV), Coxsackie virus, Hepatitis C virus, Cytomegalovirus (CMV), Human herpesvirus 6 (HHV-6), Retroviruses, and genetic factors. The incidence of<unk>  is approximately 4 per 100,000 individuals worldwide. The prevalence of<unk>  is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by<unk>  than male. The majority of<unk>  cases are reported in China, Japan, and California. Common risk factors in the development of<unk>  include family history of autoimmune diseases, serological markers such as low complement levels and cryoglobulinemia and parotid gland enlargement. The symptoms of<unk>  usually develop in the 4th and 5th decade of life, and start with symptoms such as ocular and oral dryness. Common complications of<unk>  include blurred vision and corneal damage, optic neuritis, and lymphoma. Prognosis is generally good and presence of low complement level is associated with a particularly poor prognosis among patients with<unk> . The most common symptoms of<unk>  include ocular and oral symptoms. Patients with<unk>  may have a positive history of rheumatoid arthritis (RA), systemic lupus erythematous (SLE) and non-Hodgkin B-cell lymphoma. Physical examination of patients with is usually remarkable for dryness of all mucous membranes such as mouth, eyes, lips, anal and rectal. Laboratory findings consistent with the diagnosis of<unk>  include elevated erythrocyte sedimentation rate (ESR), cytopenia, the presence of anti-SSA/Ro, anti-SSB/La. Findings on an ultrasound suggestive of<unk>  are hypoechoic and inhomogeneous salivary glands, parenchymal inhomogeneity in the submandibular glands and focal or diffuse hypoechoic or anechoic foci in glands. Parotid gland CT scan may be helpful in the diagnosis of<unk>  and finding include abnormal diffuse fat tissue deposition and diffuse punctate calcification. The most commonly used tests for dry eyes of<unk>  include Schirmer's test, ocular surface staining and tear break-up time. Pharmacologic medical therapies for dry eye, dry mouth, and other sicca symptoms of<unk>  are pilocarpine, cevimeline and artificial tears. The mainstay of treatment for<unk>  is medical therapy. Surgery is usually reserved for patients with occlusion of the lacrimal puncta, salivary gland malignancy and recurrent parotitis refractory to medical management.
Sjögren's syndrome
Arthritis-dermatitis syndrome
Behcet's syndrome
Behçet's disease
MSBD syndrome
TNF receptor associated periodic syndrome
Nasu-Hakola disease
Sever's disease