Sjogren's syndrome

Primary Sjogren's syndrome 

Autoimmune disease of exocrine glands particularly salivary and lacrimal glands characterized by dry mouth (xerostomia), dry eyes (keratoconjunctivitis sicca); other organs may be affected, including CNS and PNS, skin, and mucosal surfaces of upper airways, vagina, lungs, and kidneys.  The primary pathology is lymphocytic infiltration of exocrine glands.   

San Diego criteria for diagnosis of both primary and secondary Sjogren's syndrome (1986):

Secondary Sjogren's Syndrome

Definite: All criteria for primary Sjogren's syndrome and lack of exclusions in secondary Sjogren's syndrome

Probable: All criteria excepts biopsy

Clinical features: 

Peripheral Nerve Complications of Sjogren's Syndrome:

Treatment:  No randomized placebo-controlled trials of immunotherapies in Sjögren syndrome have been conducted.  Small fiber neuropathies aremanaged primarily symptomatically, although some patients have reported improvement with IV immunoglobulin (IVIg).  Symptomatic management is also usually used in axonal sensorimotor neuropathy, although a response to immunotherapy has been reported. Dorsal root ganglionopathy tends to be refractory to immunotherapy.  Mononeuritis multiplex with documented vasculitis is treated with high-dose corticosteroids, rituximab, or cyclophosphamide.

The classification of Sjögren syndrome applies to any individual who meets the inclusion criteria, does not have any of the conditions listed as exclusion criteria, and has a score of 4 or more when the weights from the five criteria items below are summed. 

These inclusion criteria are applicable to any patient with at least one symptom of ocular or oral dryness, defined as a positive response to at least one of the following questions: 

Exclusion criteria include prior diagnosis of any of the following conditions, which would exclude diagnosis of Sjögren syndrome and participation in Sjögren syndrome studies or therapeutic trials because of overlapping clinical features or interference with criteria tests: history of head and neck radiation treatment, active hepatitis C infection (with confirmation by polymerase chain reaction [PCR]), acquired immunodeficiency syndrome (AIDS), sarcoidosis, amyloidosis, graft versus host disease, or IgG4-related disease.

2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome 

The final classification criteria are based on the weighted sum of five items: anti-SSA/Ro antibody positivity and focal lymphocytic sialadenitis with a focus score of ≥1 foci/4 mm2, each scoring 3; an abnormal Ocular Staining Score of ≥5 (or van Bijsterveld score of ≥4), a Schirmer's test result of ≤5 mm/5 min and an unstimulated salivary flow rate of ≤0.1 mL/min, each scoring 1. Individuals with signs and/or symptoms suggestive of SS who have a total score of ≥4 for the above items meet the criteria for primary SS. Sensitivity and specificity against clinician-expert—derived case/non-case status in the final validation cohort were high, that is, 96% (95% CI92% to 98%) and 95% (95% CI 92% to 97%), respectively. 

The minor salivary gland (MSG) tissue can be diagnostically examined by using the focus score (FS) which describes the number of cell infiltrates of at least 50 mononuclear cells within 4 mm2 (i.e., a focal infiltrate). 

In practice however any 3 out of 4 criteria is used for the diagnosis of Sjogren syndrome: