The Rheumatologist: Systemic Sjogren's

I thought this article in the November 2014 issue of The Rheumatologist was pretty good. For all those sjoggies who encounter healthcare providers that claim that Sjogren's syndrome is "just dry eye and mouth", I would suggest printing this article using a very large font and shoving it.......um.......in their faces. I have put up a few excerpts in this post but by all means read it in it's entirety here.

Systemic Sjögrens: More Than a Sicca Disease 

by Pilar Brito-Zerón, MD, PhD, and Manuel Ramos-Casals, MD, PhD, on behalf of the EULAR-SS Task Force Group 

Sjögren’s syndrome (SS) is a systemic autoimmune disease in which immune-mediated inflammation causes secretory gland dysfunction, leading to dryness of the main mucosal surfaces and systemic organ involvement.1 The disease has a difficult-to-pronounce name due to the Swedish ophthalmologist Henrik Sjögren (1899–1986), who described the main disease characteristics in his 1933 doctoral thesis “Zur Kenntnis der keratoconjunctivitis sicca.” The cause of SS is unknown, but genetic and environmental factors seem to play a role. When symptoms appear in a previously healthy person, the disease is classified as primary SS (pSS). When sicca features are found in association with another systemic autoimmune disease, most commonly rheumatoid arthritis (RA), systemic sclerosis (SSc), or systemic lupus erythematosus (SLE), it is classified as associated SS; pSS may also be associated with various organ-specific autoimmune diseases.....

This infographic is excellent:

Figure 1: The spectrum of primary Sjögren’s syndrome extending from sicca syndrome and general features to systemic Sjögren’s and B-cell lymphoma.

The authors go on to define three clinical presentations of Sjogren's:

Clinical Presentation: More Than Sicca Features 

SS may be expressed in many guises, and there are three predominant clinical presentations at onset. 

The triad of dryness, fatigue & pain 

A large percentage of patients present with a clinical pattern totally dominated by severe dryness, fatigue and pain, which are not life threatening, but have a serious impact on the quality of life (see Figure 1). Oral and ocular dryness are key to the diagnosis of SS, because they occur in more than 95% of patients, although other sicca symptoms are also frequent, including hoarseness, non-productive cough, cutaneous dryness and dyspareunia in women. In addition to sicca symptoms, generalized pain and/or chronic fatigue are reported in more than 80% of patients with pSS. Physicians should be alert to women reporting dramatic quality-of-life changes due to the abrupt onset of these symptoms. However, a careful assessment is essential in these patients, because this set of symptoms is also characteristic of other processes (e.g., hypothyroidism, neoplasia, primary depression) and, above all, functional somatic syndromes, such as fibromyalgia.1 Greater intensity of dryness, fatigue and pain seems to go in tandem with less systemic involvement and identification of immunological SS features.3 A large percentage of patients present with a clinical pattern totally dominated by severe dryness, fatigue & pain. 

Nonspecific systemic inflammatory syndrome 

Some patients, especially children and the young, may present with a clinical pattern characterized by continued, well-tolerated fever, along with night sweats, fatigue, malaise and weight loss. In these patients, the lack of local symptoms and negligible or absent sicca symptoms may delay the diagnosis. However, a history of swelling of the major salivary glands (i.e., parotid and submandibular glands) may be a key feature. Laboratory tests may show normocytic anemia, mild leukopenia and, especially, a very raised erythrocyte sedimentation rate due to high serum gammaglobulin levels. Systemic infections and lymphoma should always be ruled out in these patients. 

The occult Sjögren’s syndrome (non-sicca onset) 

Some patients with pSS may present with systemic features unrelated to involvement of the mucosal surfaces.3 A large number of non-sicca features may appear before the development of sicca symptoms, including extraglandular manifestations involving the skin, lungs, kidneys or nervous system, together with some laboratory abnormalities, especially cytopenias, which, in some patients, may be symptomatic (e.g., hemolytic anemia, severe thrombocytopenia). Finally, one specific condition, pSS, presents indirectly: a fetal congenital heart block in the fetus of an asymptomatic pregnant woman, which leads to the discovery of underlying maternal anti-Ro antibodies. A significant percentage of these asymptomatic mothers will develop pSS. In all of these patients, a positive immunological result will lead to an early diagnosis of pSS several years before the onset of an overt sicca syndrome and will help prevent systemic complications (e.g., chronic organ damage, lymphoma) by ensuring their timely treatment.....continue reading here.

This article is targeted towards healthcare professionals so the language is technical; however I still recommend giving it a close look. I strongly agree with the authors' conclusion:

A greater understanding of the pathogenesis of systemic immune-mediated damage, along with active international collaborations to develop standardized guidelines, is critical.