360 |
|
|
|
|
P. Brito-Zerón et al. |
Table 25.8 Prevalence of thrombocytopenia in primary SS |
|
|
|||
|
|
Thrombocytopenia |
|
|
|
Author |
Year |
definition |
Patients (n) |
Thrombocytopenia n (%) |
|
|
|
|
|
|
|
Bloch et al. [7] |
1965 |
<150,000/mm3 |
62 |
5 |
(8) |
Alexander et al. [9] |
1983 |
<150,000/mm3 |
75 |
8 |
(11) |
Ramakrishna et al. [47] |
1992 |
<150 × 109/L |
27 |
5 |
(15) |
Ramos-Casals et al. [1] |
2002 |
<150 × 109/L |
380 |
48 (13) |
|
Ramos-Casals et al. [48] |
2008 |
<150 × 109/L |
1,010 |
131 (13) |
|
Baimpa et al. [12] |
2009 |
<140,000/mm3 |
536 |
28 (5) |
|
25.3.10Eosinophilia
In studies in the 1960s, eosinophilia was reported in about one third of patients with SS (30%) [7]. However, a subsequent study that analyzed the prevalence of eosinophilia in a large cohort of primary SS patients [1] found a prevalence of 12%. Its clinical significance in primary SS is unclear.
25.3.11Thrombocytopenia
The prevalence of thrombocytopenia in patients with primary SS ranges from 5% to 15% (Table 25.8) [1, 7, 9, 12, 47, 48]. Most studies defined thrombocytopenia as a count of <150,000 platelets; however, Baimpa et al. [12], who reported a prevalence of 5%, used a low cut-off level for platelet counts (<140,000), which may explain its lower prevalence (Table 25.8).
Thrombocytopenia is usually mild [1, 7, 9, 47, 83] and, like other hematologic abnormalities, may be the first sign of underlying SS [52, 102], either primary or associated. Thrombocytopenia in SS patients seems to be caused by peripheral platelet destruction, mediated by immune complexes or antiplatelet antibodies, with a pathogenic mechanism similar to that described in SLE patients [103]. Thrombocytopenia has been associated with anti-Ro/SS-A and anti-La/SS-B antibodies. Ramos-Casals et al. [48] reported a higher prevalence of anti-Ro/anti-La/ SS-B in patients with thrombocytopenia, as described in previous studies [9, 83].
Severe thrombocytopenia (<50,000/mm3) is uncommon in primary SS [47, 52, 103]. A good response to corticosteroids was observed in the majority of patients, although some required immunosuppressive treatment [47]. Intravenous immunoglobulins have been shown to be successful for severe thrombocytopenia refractory to steroid treatment [104].
25.4Conclusions
Laboratory abnormalities are frequent in primary SS. A raised erythrosedimentation rate and hypergammaglobulinemia are present in around 20% of patients and are more frequently found in patients with positive autoantibodies. Serum monoclonal
25 Laboratory Abnormalities in Primary Sjögren’s Syndrome |
361 |
immunoglobulins are also frequently detected (20%), with IgG being the most frequent type of monoclonal spike and k the most frequent light chain. Monoclonal bands are associated with extraglandular involvement, cryoglobulinemia, and B-cell lymphoma. b2-microglobulin may be useful in differentiating patients with sicca syndrome from those with primary SS, who often have higher serum levels, and has also been associated with extraglandular involvement and lymphoma.
Cytopenias are key laboratory abnormalities in primary SS. Although they have no clinical significance in most cases, they may be the first sign of underlying primary SS. Anemia and leukopenia are the most common hematological abnormalities reported. Anemia is usually mild, normocytic and normochromic, and is principally related to primary SS itself. Other infrequent causes of anemia described in primary SS include autoimmune hemolytic anemia, aplastic anemia, pure red cell aplasia, myelodysplastic syndrome, and pernicious anemia. Cytopenias are more frequently found in patients with positive autoantibodies and have been related to the presence of extraglandular manifestations. Recently, lymphopenia (at the expense of the CD4+ T-lymphocyte count) and neutropenia have been associated with the development of lymphoproliferative diseases. Although the spectrum of laboratory abnormalities in primary SS is wide, early detection may help the physician to identify subsets of patients who may be at risk of more severe disease.
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Part III
Diagnosis and Prognosis
Chapter 26
Diagnostic Procedures (I):
Ocular and Oral Tests
Gabriela Hernández-Molina, Francisco Cárdenas-Velazquez,
Claudia Recillas-Gispert, and Jorge Sánchez-Guerrero
Contents
26.1 |
Definitions .................................................................................................................... |
371 |
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26.2 |
Questionnaires............................................................................................................. |
371 |
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26.3 |
Ocular Tests ................................................................................................................. |
371 |
|
|
26.3.1 |
Schirmer Test .................................................................................................. |
373 |
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26.3.2 |
Vital Dyes........................................................................................................ |
374 |
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26.3.3 |
Rose Bengal .................................................................................................... |
374 |
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26.3.4 |
Fluorescein ...................................................................................................... |
375 |
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26.3.5 |
Lissamine Green ............................................................................................. |
375 |
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26.3.6 |
Tear Break-Up Time........................................................................................ |
375 |
|
26.3.7 |
Tear Osmolarity............................................................................................... |
377 |
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26.3.8 |
Tear Meniscus ................................................................................................. |
377 |
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26.3.9 |
Tear Proteins.................................................................................................... |
377 |
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26.3.10 |
Ferning Test..................................................................................................... |
377 |
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26.3.11 |
Ocular Cytology.............................................................................................. |
378 |
26.4 |
Oral Tests ..................................................................................................................... |
378 |
|
|
26.4.1 |
Wafer Test ....................................................................................................... |
379 |
|
26.4.2 |
Whole Saliva Flow Collection ........................................................................ |
379 |
|
26.4.3 |
Saxon Test ....................................................................................................... |
379 |
|
26.4.4 |
Iodine-Starch Reaction.................................................................................... |
380 |
|
26.4.5 |
Impression Cytology ....................................................................................... |
380 |
26.5 |
Conclusion ................................................................................................................... |
380 |
|
References.............................................................................................................................. |
. |
380 |
|
G. Hernández-Molina • J. Sánchez-Guerrero (*)
Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
F. Cárdenas-Velazquez • C. Recillas-Gispert
Ophthalmology Service, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
M. Ramos-Casals et al. (eds.), Sjögren’s Syndrome, |
369 |
DOI 10.1007/978-0-85729-947-5_26, © Springer-Verlag London Limited 2012 |
|
370 |
G. Hernández-Molina et al. |
Table 26.1 Conditions associated with eye and mouth dryness
Eye
Meibomian gland deficiency Local infection
Neurologic defects: V or VII paralysis
Eyelid defects: ectropion, hyperthyroidism, proptosis Contact lens use
Deficient nourishment: hypovitaminosis, A-avitaminosis
Conjunctival cicatrization: Stevens–Johnson syndrome, ocular pemphigoid, trachoma Mouth
Dehydration Mouth breathing
Hypoplasia or aplasia of salivary glands Both
Irradiation head and neck therapy Surgical glands excision Uncontrolled diabetes mellitus
Liver diseases, graft-vs.-host disease, sarcoidosis, tuberculosis, HIV or hepatitis C infection, amyloidosis
Medications
Antihistamines: pseudoephedrine, chlorpheniramine, diphenhydramine Blood pressure drugs: b-blockers, a-blockers, diuretics Antidepressants: amitriptyline, nortriptyline, imipramine, and others
Muscle relaxants: cyclobenzaprine, methocarbamol Urologic drugs: oxybutynin, bethanechol Parkinson drugs: carbidopa, levodopa, biperiden Anti-arrhythmic: disopyramide, mexiletine
Neuroleptics
Sedatives and hypnotics Anticholinergics: atropine
Primary Sjögren’s syndrome (SS) is a clinical disease characterized by keratoconjunctivitis sicca (KCS) and xerostomia due to an autoimmune background. Although the presence of one or both components is important for the diagnosis, these clinical features are not exclusively present in this syndrome.
For instance, in the general population, the prevalence of xerostomia varies between 6% and 29% [1] and of dry eye between 16% and 50% [2]. Among patients attending a rheumatologic clinic, the prevalence of dry eye and dry mouth symptoms has been reported as 45% and 43%, respectively [3]. In contrast, in primary SS patients, the prevalence of xerostomia and xerophthalmia is as high as 96% [4]. Patients with sicca syndrome usually describe a burning or foreign body sensation, itching, inability to tear, photophobia, intermittent mild visual disturbances, oral dryness, increased need of drinking water, difficulties chewing dry food, speech difficulties, and other symptoms [5]. Because there are multiple other causes of dry mouth and dry eye – particularly medications (Table 26.1) – clinicians must be certain to consider other etiologies, as well [6].