Introduction- sjögren’s syndrome
American College of Rheumatology Classification Criteria for Sjögren’s Syndrome: A Data-Driven, Expert Consensus Approach in the SICCA Cohort.
Living with Sjögren’s syndrome Sjögren’s syndrome is a long-term condition that does not usually get better on its own, although the symptoms can often be treated.
For some people it may just be a bit of a nuisance, while for others it can have frustrating long term impacts.
What is sjogren’s syndrome?
Sjögren syndrome (SS) belongs to a group of connected tissue disorders.
Dryness of the mucosa & eyelids are commonly recognized signs resulting from this autoimmune diseases.
This is usually two types of disease :
- primary Sjögren syndrome (ppSS) and
- secondary Sjögren syndrome (sSS).
Diagnosis of primary sjögren’s syndrome are defined based on formally defined classification criteria and exclude disorders which may be similar to primary sjögren’s syndrome.
Secondary Sjogren’s syndrome can also be found if symptoms of SS coexist with other systolic connective tissue diseases.
Abstract- sjögren’s syndrome
Dryness of eyes and mucosal mucosis are recognized symptoms of Sjögren syndrome (SS).
Secondary sjögren’s Syndrome ( SS ) can exist in a system where symptoms of sjögren’s syndrome are combined with other systemically related tissue problems.
The sjögren’s syndrome is often identified by a dry symptom in a combination with other connective disease and this can make identifying sjögren’s syndrome very subjective.
This blog was intended to summarize existing and future criteria for diagnosing sjögren’s syndrome.
Classification criteria for the Standards sjögren’s syndrome have not been established.
Diagnoses of sjögren’s syndrome depend upon sjögren’s syndrome‘s own experiences in research.
Sjögren’s syndrome symptoms
The symptoms of Sjögren’s syndrome include:
- dry eyes
- dry mouth
- dry skin
- tiredness
- vaginal dryness
- muscle or joint pain
- swelling between the jaw and ears (swollen salivary glands)
- rashes (especially after being in the sun).
Who is at risk for sjögren’s syndrome?
An estimated one to four million Americans have sjögren’s syndrome
Sjögren’s syndrome epidemiology
Sjögren’s Syndrome is the second most commonly reported disease in the U.S. after Systemic Lupus Erythematosus (SLE).
Sjögrens syndrome affects between 14% and 43% of the population.
Those large ranges are partly attributed to an insufficient number of diagnostic tests.
Comparisons between different ethnic groups and other international organizations have indicated that the Sjögren’s syndrome is a homogeneously inherited disorder, affecting around 1 to 1 million people.
Sjögrens syndrome has a gender ratio of 9: 1 primarily affecting women.
Sjögren’s syndrome can affect any aging person, but it usually occurs to the older person.
Onset usually occurs in 4 – 5 years.
Classification of sjögren’s syndrome
Primary
Primary Sjögren’s syndrome is where you do not have any other related conditions.
Primary Sjögren’s syndrome develops on its own, not because of any other health condition.
Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome (SS):
The classification of primary SS applies to any individual who meets the inclusion criteria, a does not have any of the conditions listed as exclusion criteria, and has a score of ≥ 4 when the weights from the five criteria items below are summed.
Secondary
There is hardly any distinct classification criteria for sjögren’s syndrome.
Previously all guidelines merely mentioned sjögren’s syndrome criteria which were not designed to be validated on a large group of sjögren’s syndrome patients.
There are 12 classification criteria to SS in 2013.
Currently classification criteria have been adopted by the American College of Rheumatology (ACR) and the European League Against Radiation.
Secondary Sjögren’s syndrome develops in addition to other autoimmune diseases like rheumatoid arthritis , lupus and psoriatic arthritis .
Table I
In order for Sjögren’s syndrome to be assessed a person has to meet the preliminary test criteria consisting of dryness of the eyes or mucous membrane.
Drying symptoms typical of sjögren’s syndrome are analyzed by questioning duration and severity.
These are the suggestions by the American-European consensus group for the year 2000 – 2002.
Table II
The domains that were in the 1995 classification criteria were first to consider domain importance. this indicates that the different symptoms in each domain have different meaning.
All sjögren’s syndrome symptoms are characterized by the presence of a specific antinuclear antibody or the presence of histopathological findings in the salivary glands.
All symptoms were given 3 points.
The 2016 diagnosis criteria are referred to in one article, but only one sentence pertains to sjögren’s syndrome.
Diagnostic methods- sjögren’s syndrome
The study used the following words to analyze the secondary sjögren syndrome and classification criteria for this study.
For a primary Sjögren’s syndrome diagnosis: Any 4 of the 6 criteria, must include either item IV (Histopathology) or VI (Autoantibodies).
Any 3 of the 4 objective criteria (III, IV, V, VI) For a secondary Sjögren’s syndrome diagnosis: In patients with another well-defined major connective tissue disease, the presence of one symptom.
Diagnosis of sjogren’s syndrome, the following criteria must be met:
I. Ocular Symptoms (at least one) Symptoms of dry eyes for at least 3 months A foreign body sensation in the eyes. Use of artificial tears 3 or more times per day
II. Oral Symptoms (at least one) Symptoms of dry mouth for at least 3 months
Recurrent or persistently swollen salivary glands
Need for liquids to swallow dry foods
III. Ocular Signs (at least one) Abnormal Schirmer’s test, (without anesthesia; ≤5 mm/5 minutes) Positive vital dye staining of the eye surface .
During an eye exam , your doctor will examine the cornea , the clear part of the eye, for dryness.
IV. Histopathology Lip biopsy showing focal lymphocytic sialoadenitis (focus score ≥1 per 4 mm 2 )
V. Oral Signs of dry mouth.
At least 2 out of the following 3:
1. Positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and anti-nuclear antibody ≥ 1:320)
2. Keratoconjunctivitis sicca with ocular staining score ≥ 3
3. Presence of focal lymphocytic sialadenitis with focus score ≥ 1 focus/4 mm 2 in labial salivary gland biopsies.
Exclusion criteria: past head and neck radiation treatment, hepatitis C infection, acquired immunodeficiency disease, preexisting lymphoma, sarcoidosis, graft versus host disease, use of anticholinergic drugs (since a time shorter than 4-fold the half-life of the drug), and IgG4 related.
Interestingly, patients suffering from limited systemic sclerosis presented with labial salivary gland infiltration with mononuclear cells in as many as 60% of patients.
Major salivary gland enlargement occurs in 25–66% of patients with primary sjögren’s syndrome.
Focus score:
Focus score of focal lymphocytic sialadenitis in labial salivary gland biopsy.
TBUT:
Tear break-up time 4 OSS:
Ocular staining score
SICCA preliminary criteria defined as at least 2 out of 3 of the following 3 objective tests:
labial salivary gland biopsy with focal lymphocytic sialadenitis and focus score ≥ 1
foci/4mm 2 ;
Ocular Staining Score ≥ 3;
positive anti-SSA/B serology or [+RF and ANA ≥ 1:320].
Biopsy
Clinical characteristics and biopsy accuracy in suspected cases of Sjögren’s syndrome referred to labial salivary gland biopsy.
- minor salivary gland biopsy + + + + 5 – salivary gland involvement
- Salivary scintigraphy + + 5 – salivary gland involvement
- Parotid sialography + + 5 – salivary gland involvement
- Unstimulated salivary secretion ≤ 0.1 ml/min + + + 6 –
- immunological anti-SSA antibodies + + + + 6 – immunological anti-SSB antibodies
Histopathology:
In a minor salivary glands (obtained through normal appearing mucosa) focal lymphocytic sialoadenitis, evaluated by an expert histopathologist, with a focus score ≥ 1, defined as number of lymphocytic foci (which are adjacent to normal-appearing mucous acini and contain more than 50 lymphocytes) per 4 mm 2 of glandular.
Imaging tests: These include sialometry, which measures how much saliva you produce by using X-rays that can see dye injected into salivary glands.
There is also salivary scintigraphy, a way to track how long it takes for a radioactive isotope to travel from an injection point in your vein to your salivary glands.
Etiology of sjögren’s syndrome
Sjögren’s Syndrome can occur as a primary inflammatory condition resulting in the exocerebro-gynecrine dysfunction.
These primary types appear with the same frequency, although the sica complex causes more severe symptoms for primary forms.
It is possible to involve most organs at the same time.
The disease affects the eyelids, the ovaries, and the parotid gland.
The epidemiological basis of Sjögren’s syndrome remains unknown.
Dryness
Approximately 20 percent of rheumatologists say there is subjective dryness in their skin.
Nonetheless, fewer than 5% meet SS standards.
The antigen SSB was rarer than other SS groups.
The xerostomia syndrome, a symptom commonly occurs with patients with systemic sclerosis.
In fact, patients exhibiting limited systemic sclerosis had infiltrated a laboratory saline gland in 60% of cases. 27% of all people have primary Sjogren’s Sydrome.
Antibodies
Anti-SSA antibodies can be detected in SS. They also exist for rheumoid arthritis and lupus erythematoses.
This implies that a classification SS could have an impact, but doesn’t confirm this.
Histopathologic analysis of the salivary gland is the only objective test to determine the presence of SS.
Practice essentials for sjögren’s syndrome
Sjögren syndrome is a systemic chronic inflammation disorder involving lymphocytose and lymphocytoses that invade exocerns.
Most commonly a woman’s symptoms occur, the median aging for the onset range is between 50 – 60 years.
Most people who have Sjögren syndrome experience Sicca syndrome symptoms including xerophthalmia or dry eye.
The sjögren syndrome is the most common cause of bilateral parotid growth.
Sicca syndrome is often seen among medical students.
Oral symptoms:
A positive response to at least one of the following questions:
1. Have you had a daily feeling of dry mouth for more than 3 months?
2. Have you had recurrently or persistently swollen salivary glands as an adult?
3. Do you frequently drink liquids to aid in swallowing dry food?
Ocular signs – that is, objective evidence of ocular involvement defined as a positive result for at least one of the following two tests:
- Schirmer’s test, performed without anesthesia (≤ 5 mm in 5 minutes)
- Tear Breakup Test
Typical questions involving:
Have you had recurrently or persistently swollen salivary glands as an adult? + + 2 –
Oral Do you frequently drink liquids to aid in swallowing dry food? + + 3 –
Ocular signs Schirmer test ≤ 5 mm in 5 minutes + + + in at least 1 eye 3 –
Ocular signs Rose Bengal score (≥ 4 according to van Bijsterveld’s scoring system) + + + in at least 1 eye 3 – ocular signs
Have you had a daily feeling of dry mouth for more than three months?
Do you have to wake up at night to drink water because your mouth is so dry?
Do you frequently drink liquids to aid in swallowing dry food?
Have you had recurrently or persistently swollen salivary glands as an adult?
Physical examination — Patients should undergo a thorough physical examination, with particular attention to the presence of salivary gland enlargement and signs of salivary hypofunction, including caries at the cervical and incisal portions of the teeth, a hyperlobulated tongue with red spots or bumps.
Conclusions
Summarizing the classification criteria for sSS, it should be emphasized that we are not currently in possession of universally accepted standards.
The most recently proposed criteria require validation in patients with sSS.
The diagnosis of sSS still depends on the experience of the investigator.
However, sSS criteria are necessary for insurance coverage.
Lastly, we recommend that all labial saliva gland tests should be performed in any case where we think SS is necessary for confirmation.
References
American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol . 2017 Jan;69(1),35-45.
Prevalence of primary sjögren’s syndrome in a US population-based cohort . Arthritis Care Res (Hoboken) . 2017 Oct;69(10):1612-1616. doi:10.1002/acr.23173.
Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002; 61:554-558
American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Ann Rheum Dis. 2017 Jan;76(1)9-16.
Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis . 2002 Jun. 61(6):554-8.
Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002; 61:554-558
Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren’s syndrome. Ann Rheum Dis . 2011 Aug. 70(8):1363-8. [QxMD MEDLINE Link] . [Full Text]
American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 2012;64:475–487. 6
