Page 768 - Clinical Immunology_ Principles and Practice ( PDFDrive )

740 ParT Six Systemic Immune Diseases

Patients presenting with sicca symptoms suggestive of SS The reasons for this increased interest mainly stems from recogni-
should undergo specialized testing to confirm the diagnosis. tion of the master regulatory roles they play in normal and
Lachrymal function can be assessed by using the Schirmer test altered physiological states and also their stability compared with
to quantify the amount of tear production, whereas corneal and mRNAs, a characteristic that allows them to be readily isolated
conjunctival damage caused by dryness can be measured by from archived samples, such as formalin-fixed paraffin-embedded
fluorescein and lissamine green staining. Salivary gland evaluation (FFPE) tissues or frozen saliva. 32-34
is done by collection of unstimulated saliva or by performing The role of miRNAs in primary SS is being investigated by
salivary scintigraphy. Laboratory workup should include testing several groups worldwide not only for their effects on immune
for autoantibodies to SSA/Ro and SSB/La antigens, which are cells but also on the target organs, such as the salivary glands. 35-37
detected in sera of 45–70% and 20–50%, respectively, in patients Rheumatoid factor is commonly positive in patients with SS
with SS. However, the AECG criteria have been criticized for and is part of the new ACR criteria for the diagnosis of SS.
including subjective symptoms and physiological tests lacking Recently, anticentromere antibodies were found to be present
specificity and for using alternative tests that are diagnostically in up to 13% of patients with SS.
nonequivalent. In addition, with the potential use of biological
agents to treat SS, more standardized, clear, and highly specific TREATMENT
criteria are needed.
To address these concerns, in 2012 the American College of
Rheumatology (ACR) proposed new classification criteria for THEraPEUTiC PriNCiPLES
SS, which required the presence of two out of three objective Management of Sjögren Syndrome (SS)
criteria in individuals suspected of having SS based on their
signs and symptoms (Table 54.1). These new criteria include no Ocular Manifestations
subjective ocular and oral symptoms and no functional tests for • Symptomatic relief with artificial tear drops and lubricating ointments
the salivary glands. • Topical cyclosporine eye drops
In subsequent studies comparing the AECG and ACR criteria, • Blockage of nasolacrimal duct by punctual plug placement or surgical
the degree of concordance ranged from modest (κ = 0.53) to cautery
high (κ = 0.81), but neither set of criteria has been shown to be
clearly superior to the other. At present, it appears that the stricter Oral Manifestations
ACR criteria are more suitable for high-risk clinical trials, such • Symptomatic relief with water and artificial saliva preparations
• Physical measures to increase saliva production: sugar-free candies
as those using biologicals, and the AECG criteria may have broader and gum
applicability in clinical research. 29-31 • Maintaining meticulous oral hygiene; use of fluoride toothpaste
• Sialogogues: pilocarpine or cevimeline
Novel Biomarkers for Diagnosis and Management of
Sjӧgren Syndrome Systemic Manifestations
MicroRNAs (miRNAs) are small regulatory noncoding RNAs • Hydroxychloroquine most commonly used to alleviate fatigue and
of 18–25 nucleotides in length. Over the last few years, interest arthralgias
in miRNAs as clinical biomarkers has been increasing steadily. • Other immunosuppressive medications may be considered in more
severe internal organ involvement
• Anti–tumor necrosis factor (TNF) blocking agents are not recommended
• Role of rituximab needs clarification
TABLE 54.1 Proposed american College of Patient Education
rheumatology (aCr) Classification Criteria • Patients should receive the following instructions:
for Sjögren Syndrome • Avoid extreme dry environments, such as proximity to open fire
places.
The classification of Sjögren syndrome (SS), which applies to • Wear protective eye glasses or goggles.
individuals with signs/symptoms that may be suggestive of SS, will • Use sugar-free sour candies or gum.
be met in patients who have at least two of the following three • Maintain good ocular and oral hygiene.
objective features: • Avoid use of medications and substances causing sicca symptoms.
1. Positive serum anti-SSA (Ro) and/or anti-SSB (La) or (positive • Women of child-bearing age with positive anti-SSA or anti-SSB antibod-
rheumatoid factor and antinuclear antibody [ANA] ≥ 1 : 320) ies should be counseled about the risk of congenital heart block.
2. Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis • Educate patients about increased risk of lymphoma associated with
with a focus score ≥1 focus/4 mm 2 SS.
3. Keratoconjunctivitis sicca with ocular staining score ≥3 (assuming • Safety precautions should be taken with regard to smoke and gas
that the patient is not currently using daily eye drops for glaucoma, leakage in the environment of patients with loss of smell.
and has not had corneal surgery or cosmetic eyelid surgery in the • Information regarding focus groups, such as Sjögren’s Syndrome
last 5 years) 3 Foundation (http://www.sjogrens.com/) and the British Sjögren’s
Prior diagnosis of any of the following conditions would exclude Syndrome Association, should be provided to patients.
participation in SS studies or therapeutic trials because of
overlapping clinical features or interference with criteria tests:
History of head and neck radiation treatment
Hepatitis C infection Symptomatic Treatment of Sicca Symptoms
Acquired immunodeficiency syndrome The mainstay of treatment in SS is providing symptomatic relief.
Sarcoidosis Use of artificial tears is often helpful. In patients with sticky
Amyloidosis mucus or strands over the eyes, use of mucolytic agents is
Graft-versus-host disease
Immunoglobulin G4 (IgG4)–related disease indicated. Blockage of lachrymal ducts with punctual plugs or
surgically can be beneficial.