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«Robert I. Fox, M.D., Ph.D. Affiliation: Rheumatology Division Scripps Memorial Hospital La Jolla, CA 92037-1220 RobertFoxMD Carla M. Fox, ...»

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PEARLS OF WISDOM (edited by John Stone)

Chapter:

Pearls of Wisdom and Myths

Regarding Sjögren’s Syndrome

Robert I. Fox, M.D., Ph.D.

Affiliation:

Rheumatology Division

Scripps Memorial Hospital

La Jolla, CA 92037-1220

RobertFoxMD@mac.com

Carla M. Fox, R.N.

CarlaFoxRN@mac.com

Affiliations:

Rheumatology Clinic

XIMED Medical Partnership

9850 Genesee Ave. Suite #910

La Jolla, CA 92037-1220 1

Abstract

(electronic version only)

Sjögren’s (“SHOW-grins”) Syndrome (SS) is manifested by:

• *keratoconjunctivitis sicca • *xerostomia • *characteristic auto antibodies including anti-nuclear antibody (ANA) and anti-Sjogren’s syndrome A (SS-A) antibody.

Pathologically, biopsies of the lacrimal and salivary glands exhibit:

• focal infiltrations of *lymphocytes,

• changes in the vascular endothelial cells,

• alteration in glandular (acinar and ductal) elements, and

• disorganization of the extra cellular matrix of the glands.

The frequency of primary SS has been a source of debate during the past decade, based on the lack of a uniform set of criteria. According to the current American-European consensus criteria, it affects approximately 0.5% of women with peaks in the age groups of 25-35 yrs and in the 50- 60 yr group. Although it also may occur in males, the female:male sex predominance is 9:1.

Myths and Pearls regarding SS generally fall into several groups:

2

• Diagnosis of disease and the relationship of SS to SLE, demyelinating disorders or fibromyalgia

• Laboratory Manifestations that reflect the difference between sensitivity and specificity of both anti-nuclear antibodies (ANA) and minor salivary gland biopsies

• Clinical Manifestations, particularly chronic fatigue and other neuropathic features, that occur in a patient with a positive ANA

• Pathologic-Clinical correlations that consider SS to be a simple deficiency of aqueous secretions of eye and mouth. In fact, the lubricating and protective capacity of tears and saliva depends on (a) mucin, (b) proteins, and (c) lipids. Further, SS patients have a higher incidence of lymphocytic infiltrative disorders, including lymphoma.

*KEYWORDS:

• ACR: American College of Rheumatology

• Auto antibodies: including anti-nuclear antibody (ANA) and anti- Sjogren’s syndrome A (SS-A) antibody

• Focal infiltration: dense substances, such as pus, blood, water, or tissue that fills alveolar spaces

• Lactimal gland: small almond-shaped structure located just above the distal corner of the eye that produces tears 3

• Lymphocytes: white blood cells that play a large role in defending the body against disease the body against disease

• Keratoconjunctivitis sicca: dry eyes

• Mucin: the inner most layer of tear film that serves as a lubricating aid. It is secreted by goblet cells that are on the surface of the eye. The mucin provides a protective barrier for the surface and serves as a means for the aqueous layer to adhere to the surface of the eye.

o Mucins produced by salivary glands play an important role in oral health by coating the tooth surface and by acting as a

–  –  –

• RA: Rheumatoid Arthritis: a chronic, systemic autoimmune disorder that causes the immune system to attack the joints, causing inflammation (arthritis), and some organs, such as the lungs and skin.

It can be a disabling and painful condition, which can lead to substantial loss of functioning mobility due to pain and joint destruction.

• Sicca symptoms: dry mouth and eyes

• SLE: Systemic Lupus Erythematosus

• SS: Sjögren’s Syndrome

–  –  –

both formation of the embryonic important signaling proteins involved in both vasculogenesis (the de novo formation of the embryonic circulatory system and angiogensis (the growth of blood vessels from pre-existing vasculature).

• Xerostomia: dry mouth I. Background (noting that Abstract is only in the electronic version) One of the most prevalent myths about SS is that dryness results from the total destruction of the glands by the immune system (discussed further below). In fact, only about 50% of the glandular elements are "destroyed" in patients with severe dryness. and the remaining ducts/acini do not function because of the inflammatory process that disrupts the ability of the residual secretory units to either release or respond to neurotransmitters such as acetylcholine (Ach) and vaso-active intestive peptide (VIP).

SS can exist as a primary condition (1*SS) or as a secondary condition (2*SS) with sicca symptoms (dry mouth and eyes)/signs in association with other well defined autoimmune disorders such as rheumatoid arthritis (RA),

–  –  –

In SS patients with florid dry eyes and dry mouth, parotid swelling and characteristic auto-antibodies, there is little debate over diagnosis.

The issue will be the extent of extraglandular involvement and the therapeutic options. In other patients, the rheumatologist may evaluate a patient referred with vague complaints of fibromyallgia and dryness, in association with the finding of a low titer ANA. In these patients, the diagnosis of SS (i.e.,, a systemic autoimmune process) is far from clear and the therapeutic decisions are more difficult.





–  –  –

SS patients may see a myriad of health care specialists (rheumatologists, hematologists, pulmonologists, cardiologists, neurologists, otolaryngologista, gastroenterologists, dentists and other oral medicine specialists, in addition to their primary care physicians (PCP’s).

–  –  –

are given conflicting information. This misinformation is often compounded by the patient’s frantic searches on the Internet, leading to retrieval of misinformation regarding diagnosis and treatment.

Instruct the patient to restrict their internet medical information searches to a much more reliable (albeit less well known) search engine "Google Scholar." http://www.googlescholar.com rather than using a more general search engine that frequently lead patients to Sjögren’s syndrome "chat groups."

–  –  –

The most time consuming and often aggravating clinical problem is the assessment of the patient with vague myalgias and chronic fatigue (i.e., Fibromyalgia).

The rheumatologist must remember that these chronic complaints are often the most significant causes of "disability" from the patient's perspective. The differential can range from depressive to demyelinating disorders, as well as toxicities from other medications or herbal supplements. The rheumatologist will need to consider central nervous system vasculitis or

–  –  –

Inform the patient with these concerns at the first visit that “fibromyalgia" represents areas of significant diagnostic and therapeutic controversy among rheumatologists.

Further, approaches to therapy must often be put off to the second revisit when further objective data is available to evaluate the correct diagnosis and disease activity. The patient can be directed to the Internet to read available

patient information on validated sites such as:

–  –  –

criteria described by Vitali et al.1, the *ACR has not yet accepted this criteria. Although the criteria have been validated in a European cohort, it has not been systematically studied in any US cohort. The European criteria are listed in Table 1.

–  –  –

There are recent criteria for disease activity and disease damage indices.

The same EEC consortium (Vitali et al 2) that spearheaded the new consensus criteria have recently presented an activity and Disease Damage Index (Tables 2 and 3)2 that will serve as a starting point for a uniform data base basis for clinical data collection and research studies. These indices will provide the same type of standardization that the *ACR criteria provided for *RA.

–  –  –

with objective findings and the large role that fibromyalgia plays in both the physician’s and patient’s assessment of “quality of life.” The problems in generating measures for evaluating *SS are more comparable to non--renal *SLE. Indeed, the new criteria for SS later are based on similar measures in SLE agents such as SELENA and Bilag, where the absence of definitive biomarkers has made disease assessment difficult.

It should also be noted that the new measures extend the work of Bowman et al3 in the development of a clinical disease activity index3, that was acknowledged as a basis for the Vitali scores quoted above.

–  –  –

“The antinuclear antibody (ANA) and anti-Sjögren’s SS-A (Ro) antibody are specific for primary Sjögren’s syndrome.” MythBuster: Patients commonly arrive in the rheumatologist's office after the primary care physician has ordered a battery of tests for vague symptoms and a positive ANA and/or a positive anti-SS A (Ro) emerged.

–  –  –

can create misunderstanding when the limitations are not fully appreciated.

The ANA has a higher sensitivity than specificity. Tan et al4 measured the range of antinuclear antibodies (ANA) in "healthy" individuals.

• Fifteen international laboratories experienced in performing tests for ANA by indirect immuno-fluorescence participated in analyzing coded sera from healthy individuals.

• Except for the stipulation that HEp-2 cells should be used as substrate, each laboratory used its own in-house methodology so that the data might be expected to reflect the output of a cross-section of worldwide ANA reference laboratories.

• The sera were analyzed at 4 dilutions: 1:40, 1:80, 1:160, and 1:320.

• They found that in healthy individuals, the frequency of ANA did not differ significantly across the 4 age subgroups spanning 20-60 years

–  –  –

An interesting finding of this study was a remarkably higher incidence of "false" positive ANA's in patients with either a monoclonal gammopathy; or

–  –  –

association of autoantibody with a "dysregulated" immune system at the bone marrow level.

Lightfoot has used Baysian calculation to determine that an individual with an ANA of 1:320 (and lacking other clinical criteria to suggest either SS or SLE) has less than a 1:100 chance in developing SLE or SS during a 5-year follow-up interval.

–  –  –

“SS Patients with "atypical antibody profiles" such as an ANA with anticentromere pattern or a ANCA always represent overlap syndromes with other conditions such as Progressive Systemic Sclerosis (PSS) or Wegener's granulomatosus (WG)”.

MythBuster: Although patients may develop an overlap syndrome with other autoimmune disorders such as PSS, the pattern of auto antibodies in patients with SS correlates more closely with their HLA-DR than with their clinical presentation5, 6.

–  –  –

atypical antibodies including anti-centromere or ANCA.

• Patients with atypical auto-antibodies had no statistical differences extraglandular manifestations (except for a higher prevalence of Raynaud's phenomenon, 28% versus 7%),

–  –  –

MRI sialography can be used to visualize the ductal structure of the major salivary glands.

It is not necessary to perform a sialogram to assess the salivary status of SS patients or to visualize the ductal structures for punctual sialadenilits. This is important since most US academic centers do not have experience in retrograde sialography that is mentioned in diagnostic criteria and this invasive method may have morbidity if done by inexperienced radiologists or ENT.

MRI imaging of the parotid and submandibular glands has vastly improved8.

If an MRI of the soft tissues of the neck is required (for example in a case of parotid gland swelling), then we ask for a gadolinium contrast study with "fat suppression" views that provides a nice evaluation of the glandular tissues9, 10. Although ultrasound of the glands has proven useful at certain

–  –  –

required to obtain reproducible results. As a result of readily available MRI imaging at most academic medical centers in the US, experience with ultrasound imaging of the glands has not been fully developed.

–  –  –

Salivary flow rates can be evaluated by non- or minimally invasive methods.

This is important to correlate measurements of patient's symptoms with objective signs of dryness. Technicium scans of salivary function are performed after coating the tongue with a lemon concentrate11-13. The uptake of contrast material and its rate of secretion into the gland can be quantitated. Although the decreased flow rate is not specific to SS (i.e., many processes can contribute to decreased uptake or secretion), the method is useful in the evaluation of the patient who complains that "I don’t feel any saliva in my mouth," but the oral mucosal tissues appear to be relatively intact. The finding of a normal Technicium scan should point the rheumatologist towards other causes of the patient's severe mouth complaints.

–  –  –

in whom a diagnosis of SS could not be supported, even though they reported symptoms of dryness and taste disturbance. The authors suggested a local neuropathy or even psychogenic causes.

–  –  –

There is significant variation when collecting saliva by oral expectoration or "sponge" methods14, 15.

Simple expectorated saliva can be collected on a pre-weighed sponge placed under the tongue (called the Saxon test)16. However, there is significant variability in these measurements in the same patient over the course of the day or when measurements are repeated14. The reasons for the variability

include:



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