311Diagnosis
The diagnosis of SS is often challenging, hence it is not infrequently overlooked.
Initially, the diagnosis requires a high index of suspicion with a focused enquiry
about dry eyes and dry mouth. A thorough history and clinical scrutiny for extraglan-
dular affection is also mandatory. Without a high index of suspicion, the nonspecifi c
nature of symptoms may lead to a considerable delay of the diagnosis that averaged
7 years in one survey [ 10 ]. Tests to quantify lacrimal (e.g., Schirmer test) and salivary
(e.g., sialometry) secretions are utilized to diagnose KCS and dry mouth [ 1 ]. Salivary
gland ultrasonography, magnetic resonance angiography (MRA), and biopsy are also
used in the diagnostic evaluation of dry mouth [ 1 , 11 ]. Several non-specifi c labora-
tory markers of SS are described. These include the presence of anemia, leukopenia,
eosinophilia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate
(ESR) [ 1 ]. Testing for autoantibodies is mandatory in the workup of SS. Antibodies
to SS antigen type A (SSA), also called anti-Ro, and/or antibodies to SS antigen type
B (SSB), also called anti-La, are present in a considerable proportion of patients with
pSS. Anti-Ro and/or anti-La may also be found in some of the sSS patients and occa-
sionally in healthy individuals as well. Additionally, their absence does not rule out
the diagnosis of SS. Antinuclear antibodies may test positive in pSS and in SS sec-
ondary to SLE. Rheumatoid factor may be detected in patients with pSS and
RA-associated sSS as well. The diagnosis of SS should not be confused with other
disorders that may lead to similar systemic symptoms (e.g., fi bromyalgia and depres-
sion), glandular enlargement (lymphoma or sarcoidosis), and dry mouth (e.g. the use
of drugs with anticholinergic side effects) [ 1 , 3 ]. To improve diagnostic accuracy a
number of diagnostic classifi cation criteria have evolved to be used by professionals
and researchers. The most widely accepted set of criteria are known as the American-
European Consensus Group classifi cation criteria (AECG) [ 12 ].
Treatment Modalities
Artifi cial tears eye drops and lubricating ointments are used to treat dry eyes.
Cyclosporine 0.05 % eye drops are usually kept for severe cases. Saliva replacement
products and sugar-free chewing gums may improve the dry mouth symptoms. Oral
drugs that increase salivary secretions (sialogogues), for example, pilocarpine, were
found effective also to treat xerostomia [ 13 ]. There is limited or no evidence for the
benefi t of systemic immunosuppressive/immunomodulatory drugs (mycophenolate,
azathioprine, and cyclosporine) in the treatment of KCS and xerostomia [ 13 ]. Studies
that evaluated the synthetic disease-modifying antirheumatic drugs (DMARDS)—
methotrexate, lefl unomide, and hydroxychloroquine—found only limited improve-
ments in sicca symptoms [ 3 , 13 ]. Trials on the tumor necrosis factor alpha inhibitors
12 PROMs for Sjögren’s Syndrome