Primary Sjogren's Syndrome - all of the following:
(1) keratoconjunctivitis sicca (see chapter on ophthalmology)
(2) xerostomia (see chapter on dentistry and oral medicine)
(3) no concurrent connective tissue disease
Secondary Sjogren's Syndrome:
(1) keratoconjunctivitis sicca and/or xerostomia
(2) connective tissue disorder
(2a) rheumatoid arthritis
(2b) Systemic Lupus Erythematosus
(2c) progressive systemic sclerosis, scleroderma
(2d) dermatomyositis, polymyositis
(2e) Primary Biliary Cirrhosis (PBC)
(2f) Wegener's Granulomatosis (WG)
(2g) Polyarteritis Nodosa (PAN)
Possible or Incipient Sjogren's Syndrome:
(1) keratoconjunctivitis sicca or xerostomia
(2) one of the following conditions associated with an immune dysfunction in the absence of a diagnosed connective tissue disorder:
(2a) pulmonary lymphocytic interstitial infiltrates
(2b) interstitial nephritis and/or renal tubular acidosis
(2c) hypergammaglobulinemia
(2d) vasculitis with purpura
(2e) polymyopathy
(2f) chronic noncirrhotic liver disease
(2g) neuropathy
