- Enlarged lacrimal glands (HP:0007734): Abnormally big lacrimal glands. Evidence: TAS. Frequency: Very frequent (HP:0040281). (ORPHA:79078)
- Abnormal salivary gland morphology (HP:0010286): Any abnormality of the salivary glands, the exocrine glands that produce saliva. Evidence: TAS. Frequency: Very frequent (HP:0040281). (ORPHA:79078)
- Abnormality of the submandibular glands (HP:0010287): Any abnormality of the submandibular glands, which are the salivary glands that are located beneath the floor of the mouth, superior to the digastric muscles. Evidence: TAS. Frequency: Very frequent (HP:0040281). (ORPHA:79078)
- Increased circulating IgG4 concentration (HP:0032300): An abnormally increased concentration of the IgG4 subtype in the blood circulation. Evidence: TAS. Frequency: Very frequent (HP:0040281). (ORPHA:79078)
- Enlargement of parotid gland (HP:0011801): Increased size of the parotid gland. Evidence: TAS. Frequency: Frequent (HP:0040282). (ORPHA:79078)
- Xerostomia (HP:0000217): Dryness of the mouth due to salivary gland dysfunction. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Facial edema (HP:0000282). Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Abnormality of the orbital region (HP:0000315). Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Proptosis (HP:0000520): An eye that is protruding anterior to the plane of the face to a greater extent than is typical. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Abnormal optic nerve morphology (HP:0000587): Abnormality of the optic nerve. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Periorbital fullness (HP:0000629): Increase in periorbital soft tissue. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Keratoconjunctivitis sicca (HP:0001097): Dryness of the eye related to deficiency of the tear film components (aqueous, mucin, or lipid), lid surface abnormalities, or epithelial abnormalities. Keratoconjunctivitis sicca often results in a scratchy or sandy sensation (foreign body sensation) in the eyes, and may also be associated with itching, inability to produce tears, photosensitivity, redness, pain, and difficulty in moving the eyelids. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Weight loss (HP:0001824): Reduction of total body weight. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Fever (HP:0001945): Body temperature elevated above the normal range. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Autoimmunity (HP:0002960): The occurrence of an immune reaction against the organism's own cells or tissues. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Increased circulating IgA concentration (HP:0003261): An abnormally increased level of immunoglobulin A in blood. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Generalized muscle weakness (HP:0003324): Generalized weakness or decreased strength of the muscles, affecting both distal and proximal musculature. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Antinuclear antibody positivity (HP:0003493): The presence of autoantibodies in the serum that react against nuclei or nuclear components. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Increased circulating IgM concentration (HP:0003496): An abnormally increased level of immunoglobulin M in blood. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Abnormality of the extraocular muscles (HP:0008049): An abnormality of an extraocular muscle. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Fatigue (HP:0012378): A subjective feeling of tiredness characterized by a lack of energy and motivation. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Cytoplasmic antineutrophil antibody positivity (HP:0032230): The presence of autoantibodies in the serum that react against proteins predominantly expressed in cytoplasmic granules of neutrophils. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Palpebral edema (HP:0100540): Edema in the region of the eyelids. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Myositis (HP:0100614): A general term for inflammation of the muscles without respect to the underlying cause. Evidence: TAS. Frequency: Occasional (HP:0040283). (ORPHA:79078)
- Blindness (HP:0000618): Blindness is the condition of lacking visual perception defined as a profound reduction in visual perception. On the 6m visual acuity scale, blindness is defined as less than 3/60. On the 20ft visual acuity scale, blindness is defined as less than 20/400. On the decimal visual acuity scale, blindness is defined as less than 0.05. Blindness is typically characterized by a visual field of no greater than 10 degrees in radius around central fixation. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Thrombocytopenia (HP:0001873): A reduction in the number of circulating thrombocytes. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Tubulointerstitial nephritis (HP:0001970): A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Lymphadenopathy (HP:0002716): Enlargement (swelling) of a lymph node. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Retroperitoneal fibrosis (HP:0005200). Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Nodular goiter (HP:0005994): Enlargement of the thyroid gland related to one or more nodules in the thyroid gland. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Optic nerve compression (HP:0007807). Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Abnormal lacrimal duct morphology (HP:0011481): An abnormality of the lacrimal duct, a duct that drain tears from the conjunctiva, via the lacrimal puncta, into the lacrimal sac. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
- Thyroiditis (HP:0100646): Inflammation of the thyroid gland. Evidence: TAS. Frequency: Very rare (HP:0040284). (ORPHA:79078)
These phenotypes are associated with the disease IgG4-related dacryoadenitis and sialadenitis (ORPHA:79078).