Sjogren's Syndrome Support Group
Sjogren's syndrome is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It also associated with rheumatic disorders such as rheumatoid arthritis, and it is rheumatoid factor positive in 90 percent of cases.
RenneG
I copied and pasted this information from this website:
http://emedicine.medscape.com/article/1066649-overview
It is worth reviewing for those odd symptoms like the ones I have experienced.
Secondary Sjgren syndrome appears late in the course of the primary disease. However, in some patients, primary Sjgren syndrome may precede systemic lupus erythematosus by many years. Secondary Sjgren syndrome is usually mild, and sicca symptoms are the main feature. Unlike those with primary Sjgren syndrome, patients with the secondary type have significantly fewer systemic manifestations. These manifestations include salivary gland swelling, lung involvement, nervous system involvement, renal involvement, Raynaud phenomenon, and lymphoproliferative disorders. In secondary Sjgren syndrome, symptoms of the primary disease predominate. Secondary Sjgren syndrome does not modify the prognosis or outcome of the basic disease.
The physical symptoms of primary Sjgren syndrome can be divided into glandular and extraglandular symptoms.
Glandular symptoms
Ocular
Keratoconjunctivitis sicca or dry eye syndrome, characterized by chronic dryness of the cornea and conjunctiva
Discomfort (eg, irritation, pain, redness, burning, itching, foreign body sensation, photophobia, blurred vision)
Mouth
Dryness
Tongue - Red, smooth, and dry
Dental caries - Severe and progressive
Parotid duct narrowing
Lips - Red, dry, and scaly
Cracks at the corners of the mouth
Chronic oral candidiasis
Periodontal conditions: These were evaluated in Sjgren syndrome patients, and Sjgren syndrome appears to negatively affect the periodontal condition. Gingival inflammation was more evident in the individuals with Sjgren syndrome, particularly those with secondary Sjgren syndrome.5
Recurrent swelling of the parotid glands (22-66% of patients), sometimes also submaxillary and sublingual glands
Other mucous membranes
Atrophic changes in the mucous membranes of the upper respiratory tract leading to nasal dryness, recurrent infections, hoarseness, and aphonia
Atrophic rhinitis
Atrophic changes in the vulva and vagina resulting in pruritus and vaginitis
Dryness of the anal and rectal mucous membranes (eg, pruritus, inflammation)
Skin
Dryness of the skin occurs in 50% of patients with Sjgren syndrome; scaling occurs in about 25% of patients. The skin may be irritable with secondary lichenification.
Partial or complete loss of sweating may be present.
Hair may be dry, sparse, and brittle; diffuse alopecia may involve the scalp, limbs, axillae, or pubis.
Nail folds may show capillaroscopic abnormalities, which are associated with the presence of antiendothelial cell antibodies.6
Erythema of the nose and cheeks may be present.
Annular erythematous rash with scales, localized especially on the face and neck, is recognized as a cutaneous manifestation of Sjgren syndrome. The lesions are recurrent and clear without pigmentation; no photosensitivity is observed.
A higher risk of cutaneous vasculitis is noted.
In Japanese patients with Sjgren syndrome, annular erythema is divided into 3 types: Sweet diseaselike annular erythema with an elevated border, subacute cutaneous lupus erythematosuslike marginally scaled erythema, and papular erythema. These lesions bear some clinical similarities to the annular lesions of subacute cutaneous lupus erythematosus, but their histopathologic features are distinct from those of subacute cutaneous lupus erythematosus. Significant mucin depositions are observed.
Sjgren vasculitis involves postcapillary venules, typically on the lower legs. These patients were formerly classified as having Waldenstrm hypergammaglobulinemic purpura.
Extraglandular symptoms
Gastrointestinal tract
Esophageal motility abnormalities
Pancreatic involvement
Splenomegaly
Digestive symptoms (due to atrophy of the gastric mucous membrane with achlorhydria)
Hepatitis (13%)
Lungs7 : Pulmonary abnormalities occur in 9-29% of cases; they are similar in both primary and secondary Sjgren syndrome.
Pulmonary fibrosis
Pulmonary hypertension
Recurrent chest infections
Granulomatous infiltration and fibrosing alveolitis
Restrictive ventilatory defect
Impaired gas transfer
Articular changes (eg, arthritis): These occur in 42% of patients with Sjgren syndrome.
Urinary tract: Patients with Sjgren syndrome have significantly more urinary problems than those without Sjgren syndrome.
Symptoms of an irritated bladder
Urinary frequency and suprapubic pain
Renal tubular dysfunction: Patients with primary Sjgren syndrome commonly are first seen because of renal impairment, usually from renal tubular dysfunction.8
Renal tubular acidosis: This affects one third of patients with Sjgren syndrome. A correlation apparently exists between hypergammaglobulinemia and distal renal tubular acidosis.8
Interstitial nephritis (This is rare; occurs in 4% of cases; and is often accompanied by cryoglobulinemia, a decreased level of complement, and the presence of circulating immune complexes.)
Impaired renal concentrating ability, generalized aminoaciduria
Nervous system
A combination of lesions and relapses can suggest multiple sclerosis. Myelopathy rarely occurs in the course of primary Sjgren syndrome. It appears as Brown-Squard syndrome, acute transverse myelitis, or progressive myelopathy. Clinically, cases with nervous system involvement present with paraparesis or paraplegia resulting from lesions at the thoracic or cervicothoracic levels.
Peripheral neuropathy occurs in 10-35% patients with primary Sjgren syndrome. Peripheral nerve dysfunction may occur; this can include trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensorimotor polyneuropathy, or pure sensory neuropathy. This tends to be a small-fiber peripheral neuropathy.9 Painful distal paresthesias in the feet may be evident, as may abnormal sweating. Examination may reveal findings that include decreased pinprick sensation.
Isolated cranial nerve involvement rarely occurs in primary Sjgren syndrome.
Central nervous system involvement is less common (10-25% of patients with Sjgren syndrome) than other types of involvement. It ranges from neuropathy, hemiparesis, transverse myelitis, and dystonia to even encephalopathy and dementia.
In Sjgren syndrome, focal brain lesions can be present in the cerebral white matter.
Dysregulation of hypothalamic-pituitary-adrenal and thyroid axes can cause some neurologic disturbances.
Causes
The following are causes of Sjgren syndrome.
Genetic factors
The presence of HLA-DRB1, HLA-DRB3, HLA-DR5, HLA-DRw11, HLA-DR52, HLA-DRw53, and other HLAs increase the risk of Sjgren syndrome.
Polymorphism of the mannose-binding lectin gene is likely to be one of the genetic factors that determines an individual's susceptibility to Sjgren syndrome.
Viral infections
Epstein-Barr virus
HTLV-1 and HIV-1
Human herpesvirus 6
Hepatitis C virus
Cytomegalovirus
Autoimmune dysregulation (loss of immune tolerance and production of various autoantibodies, eg, ANA, anti-Ro, anti-La)
Dysregulation of apoptosis
Adrenal and gonadal steroid hormone deficiency
http://emedicine.medscape.com/article/1066649-overview
It is worth reviewing for those odd symptoms like the ones I have experienced.
Secondary Sjgren syndrome appears late in the course of the primary disease. However, in some patients, primary Sjgren syndrome may precede systemic lupus erythematosus by many years. Secondary Sjgren syndrome is usually mild, and sicca symptoms are the main feature. Unlike those with primary Sjgren syndrome, patients with the secondary type have significantly fewer systemic manifestations. These manifestations include salivary gland swelling, lung involvement, nervous system involvement, renal involvement, Raynaud phenomenon, and lymphoproliferative disorders. In secondary Sjgren syndrome, symptoms of the primary disease predominate. Secondary Sjgren syndrome does not modify the prognosis or outcome of the basic disease.
The physical symptoms of primary Sjgren syndrome can be divided into glandular and extraglandular symptoms.
Glandular symptoms
Ocular
Keratoconjunctivitis sicca or dry eye syndrome, characterized by chronic dryness of the cornea and conjunctiva
Discomfort (eg, irritation, pain, redness, burning, itching, foreign body sensation, photophobia, blurred vision)
Mouth
Dryness
Tongue - Red, smooth, and dry
Dental caries - Severe and progressive
Parotid duct narrowing
Lips - Red, dry, and scaly
Cracks at the corners of the mouth
Chronic oral candidiasis
Periodontal conditions: These were evaluated in Sjgren syndrome patients, and Sjgren syndrome appears to negatively affect the periodontal condition. Gingival inflammation was more evident in the individuals with Sjgren syndrome, particularly those with secondary Sjgren syndrome.5
Recurrent swelling of the parotid glands (22-66% of patients), sometimes also submaxillary and sublingual glands
Other mucous membranes
Atrophic changes in the mucous membranes of the upper respiratory tract leading to nasal dryness, recurrent infections, hoarseness, and aphonia
Atrophic rhinitis
Atrophic changes in the vulva and vagina resulting in pruritus and vaginitis
Dryness of the anal and rectal mucous membranes (eg, pruritus, inflammation)
Skin
Dryness of the skin occurs in 50% of patients with Sjgren syndrome; scaling occurs in about 25% of patients. The skin may be irritable with secondary lichenification.
Partial or complete loss of sweating may be present.
Hair may be dry, sparse, and brittle; diffuse alopecia may involve the scalp, limbs, axillae, or pubis.
Nail folds may show capillaroscopic abnormalities, which are associated with the presence of antiendothelial cell antibodies.6
Erythema of the nose and cheeks may be present.
Annular erythematous rash with scales, localized especially on the face and neck, is recognized as a cutaneous manifestation of Sjgren syndrome. The lesions are recurrent and clear without pigmentation; no photosensitivity is observed.
A higher risk of cutaneous vasculitis is noted.
In Japanese patients with Sjgren syndrome, annular erythema is divided into 3 types: Sweet diseaselike annular erythema with an elevated border, subacute cutaneous lupus erythematosuslike marginally scaled erythema, and papular erythema. These lesions bear some clinical similarities to the annular lesions of subacute cutaneous lupus erythematosus, but their histopathologic features are distinct from those of subacute cutaneous lupus erythematosus. Significant mucin depositions are observed.
Sjgren vasculitis involves postcapillary venules, typically on the lower legs. These patients were formerly classified as having Waldenstrm hypergammaglobulinemic purpura.
Extraglandular symptoms
Gastrointestinal tract
Esophageal motility abnormalities
Pancreatic involvement
Splenomegaly
Digestive symptoms (due to atrophy of the gastric mucous membrane with achlorhydria)
Hepatitis (13%)
Lungs7 : Pulmonary abnormalities occur in 9-29% of cases; they are similar in both primary and secondary Sjgren syndrome.
Pulmonary fibrosis
Pulmonary hypertension
Recurrent chest infections
Granulomatous infiltration and fibrosing alveolitis
Restrictive ventilatory defect
Impaired gas transfer
Articular changes (eg, arthritis): These occur in 42% of patients with Sjgren syndrome.
Urinary tract: Patients with Sjgren syndrome have significantly more urinary problems than those without Sjgren syndrome.
Symptoms of an irritated bladder
Urinary frequency and suprapubic pain
Renal tubular dysfunction: Patients with primary Sjgren syndrome commonly are first seen because of renal impairment, usually from renal tubular dysfunction.8
Renal tubular acidosis: This affects one third of patients with Sjgren syndrome. A correlation apparently exists between hypergammaglobulinemia and distal renal tubular acidosis.8
Interstitial nephritis (This is rare; occurs in 4% of cases; and is often accompanied by cryoglobulinemia, a decreased level of complement, and the presence of circulating immune complexes.)
Impaired renal concentrating ability, generalized aminoaciduria
Nervous system
A combination of lesions and relapses can suggest multiple sclerosis. Myelopathy rarely occurs in the course of primary Sjgren syndrome. It appears as Brown-Squard syndrome, acute transverse myelitis, or progressive myelopathy. Clinically, cases with nervous system involvement present with paraparesis or paraplegia resulting from lesions at the thoracic or cervicothoracic levels.
Peripheral neuropathy occurs in 10-35% patients with primary Sjgren syndrome. Peripheral nerve dysfunction may occur; this can include trigeminal sensory neuropathy, mononeuropathy multiplex, distal sensorimotor polyneuropathy, or pure sensory neuropathy. This tends to be a small-fiber peripheral neuropathy.9 Painful distal paresthesias in the feet may be evident, as may abnormal sweating. Examination may reveal findings that include decreased pinprick sensation.
Isolated cranial nerve involvement rarely occurs in primary Sjgren syndrome.
Central nervous system involvement is less common (10-25% of patients with Sjgren syndrome) than other types of involvement. It ranges from neuropathy, hemiparesis, transverse myelitis, and dystonia to even encephalopathy and dementia.
In Sjgren syndrome, focal brain lesions can be present in the cerebral white matter.
Dysregulation of hypothalamic-pituitary-adrenal and thyroid axes can cause some neurologic disturbances.
Causes
The following are causes of Sjgren syndrome.
Genetic factors
The presence of HLA-DRB1, HLA-DRB3, HLA-DR5, HLA-DRw11, HLA-DR52, HLA-DRw53, and other HLAs increase the risk of Sjgren syndrome.
Polymorphism of the mannose-binding lectin gene is likely to be one of the genetic factors that determines an individual's susceptibility to Sjgren syndrome.
Viral infections
Epstein-Barr virus
HTLV-1 and HIV-1
Human herpesvirus 6
Hepatitis C virus
Cytomegalovirus
Autoimmune dysregulation (loss of immune tolerance and production of various autoantibodies, eg, ANA, anti-Ro, anti-La)
Dysregulation of apoptosis
Adrenal and gonadal steroid hormone deficiency
and they call it the dry eye disease?????...
wow how wrong that statement is?..
SS can be very serious....i see that..im left wondering why my Rhumey doesnt see it?...
what a worry..
I think ill get tested for an epstien bar ?..
.......you shouldn't be paying anyone then giving him information to learn, that's absurd! If he doesn't know about SjS then he is not a Rheumy IMHO. They know about it , but don't like to expond and make people worry.
If you want sympathy or empathy go to a self help group for SjS. Remember these articles are doctor written not everyone has the symptoms.
...anyone can google this information, a low percentage of people never get any of these manifestations. They start thinking they will get them.
I have secondary- I know first hand. You want up -to date information join : Join THE MOISTURE SEEKERS-
http://www.sjogrens.org/home/about-sjogrens-syndrome/symptoms