Sjogren’s in younger groups – Comments

Some comments from Dr David Zimmerman (Hon President)
(extract from Dec 2012 Newsletter)

Sponsorship was hard-won this year and amounts given by various firms was well down on previous years and reflects no doubt the harder financial times.  As so many members are on fixed incomes this is doubly hard as costs go up, despite the buoyancy of the Auckland property market.

Below is the abstract from a Lancet article and while the diagnostic process is one we are familiar with, I note that the  pattern of membership is changing with what seems to be a much younger  group coming on board. I suspect that these are people where the disorder starts, perhaps in puberty and manifests in mid to late 20’s, finally forcing them to seek help at 30-ish. This new variant does not show  up positive on lip biopsy. So the fall-back position is blood tests.

However the underlying issue is to find out the mechanism that drives these disorders. Speaking to Dr Chen, I did understand that there is an immunoglobulin that hides deep in the glands and is difficult to get at. This encourages the protective cells to go and dig it out thus filling the glands with unproductive cells effectively blocking the gland.

Therefore I understand the key is to find what the initial trigger is and how to switch it off.

Sjögren’s syndrome

Robert I Fox

Sjögren’s syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Dryness of the mouth and eyes results from involvement of the salivary and lacrimal glands. The accessibility of these glands to biopsy enables study of the molecular biology of a tissue-specific autoimmune process. The exocrinopathy can be encountered alone (primary Sjögren’s syndrome) or in the presence of another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. A new international consensus for diagnosis requires objective signs and symptoms of dryness including a characteristic appearance of a biopsy sample from a minor salivary gland or autoantibody such as anti-SS-A. Exclusions to the diagnosis include infections with HIV, human T-lymphotropic virus type I, or hepatitis C virus. Therapy includes topical agents to improve moisture and decrease inflammation. Systemic therapy includes steroidal and non-steroidal anti-inflammatory agents, disease-modifying agents, and cytotoxic agents to address the extraglandular manifestations involving skin, lung, heart, kidneys, and nervous system (peripheral and central) and haematological and lymphoproliferative disorders. The most difficult challenge in diagnosis and therapy is patients with symptoms of fibromyalgia (arthralgia, myalgia, fatigue) and oral and ocular dryness in the presence of circulating antinuclear antibodies.    Lancet 2005; 366: 321–31

Rheumatology Clinic, Scripps Memorial Hospital and Research Foundation, La Jolla, CA 92037, USA
(R I Fox MD)
Correspondence to: Dr Robert I Fox, Rheumatology Clinic, 9850 Genesee Ave, #910, La Jolla, CA 92037, USA

Anti-SSA (RO) is common, but is only found in 70% of patients finally diagnosed with an autoimmune disorder. Conversely  13.8% of a USA population was found to have this, far higher than Sjogren’s in the population, so for us it is helpful, but not diagnostic.  What things might add to or facilitate such a disorder.  How about smoking?  Nope! Neither is C-reactive protein, which is associated with inflammation. So this appears to be more linked to the interferon pathway.

AntiSSA (RO)  it may be helpful, but it’s not diagnostic, so that begs the questions… what is diagnostic? This illustrates the inadequacy of our diagnostic tools and therefore understanding of the disorder and a pathway to treatment.

Where to then… my fallback position is medieval. If it looks like a horse, neighs and eats hay, it’s probably a horse, so treat accordingly and watch the results. However this information was not available a few years ago. So hopefully it will lead to more answers

 With the destruction I see dentally, every step is urgent, the cost of delay and lack of control is high, financially, physically and emotionally, hence the need to focus funds on research where the best chance of a result is likely.