RHEUMATOLOGYCASE

REPORT

DEA GODERDZISHVILI
 CASE REPORT
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A 45-year-old woman with a 2-year history of increasing unsteadiness was referred

from neurology to the rheumatology department. She had a 10-year history of chronic
fatigue, arthralgia, and myalgia. She had a history of photosensitive rash. She had
stopped wearing contact lenses in her early twenties because of symptomatic dry eyes,
carried a water bottle with her to work, and had a constant irritating dry cough. Her
sister had autoimmune thyroid disease. On examination, she was obese and
normotensive, with tender but not swollen wrist, finger, and knee joints. She had
significant dental caries. She had a wide-based staggering gait and was unable to heel–
toe walk. Neurological examination was other- wise normal.
 CASE REPORT - LAB WORK
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• Hb 12.4 g/L; WCC 3.8×109/L; lymphocyte 0.87×109/L

• Liver and renal function = normal
• RF = ⊖;
• ANA = ⊕ 1:80
• Ant-Ro Antibodies = ⊕
• Lumbar puncture for oligoclonal bands were matched in CSF and serum
•  MRI scan of brain showed extensive periventricular white matter changes
in the frontal, parietal and occipital lobes
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Possible Diagnosis?Differ
 DIFFERENTIAL DIAGNOSIS
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• Sarcoidosis

• Viral Infections: HIV, Mumps, HCV

• Multiple Sclerosis?
 SJOGREN’S SYNDROME
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American-European Consensus Crit

The American–European Consensus Group (AECG) classification criteria = 4+

•  Ocular symptoms—persistent troublesome dry eyes daily for more than 3 months, or current sensation of
sand or gravel in the eyes or need for tear substitute more than three times daily.
•  Oral symptoms—daily feeling of dry mouth for >3 months, or current persistently swollen salivary glands
in adults, or frequently drinking liquids to aid swallowing dry food.
• Objective dry eyes—positive Schirmer’s Test (≤5 mm in 5 min) or positive Rose Bengal score.
• Histopathology—minor salivary gland biopsy showing focal lymphocytic sialad- enitis, ductal hyperplasia,
and acinar atrophy.
• P ositive biopsy has, on focus, >50 lymphocytes per 4 mm2 tissue. The pre- dominant lymphocyte is type
CD4 positive.
• Objective dry mouth: unstimulated whole salivary flow <1.5 mL/15 min or parot- id sialography showing
diffuse sialectasis without major duct involvement or sali- vary scintigraphy showing delayed uptake of
tracer.
• ◆  Serology: positive Ro or positive La antibodies or both.
•
 DDX: MULTIPLE SCLEROSIS
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Neurological Sjögren’s syndrome occurs in 1–5% of pSS patients.

Clinical presentation is highly variable and can affect the peripheral and central nervous systems,
cognitive function, and psychological state.

CNS lesions can be very difficult to distinguish from multiple sclerosis (MS) on MRI. MRI lesions of pSS
resemble multiple-sclerosis-like deposits, but are more diffuse.

Clinically, it can also be difficult to distinguish CSF in Sjögren’s syndrome from that in multiple sclerosis.

The main distinguishing feature is that matched oligoclonal bands are present in both CSF and blood,
whereas in multiple sclerosis they are present only in the CSF.
 TREATMENT OPTIONS
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• For dry mouth

•

• Regular dental care

• Adequate hydration and foods that stimulate salivary flow (e.g., dried
fruit slices)

• In moderate/severe disease: artificial saliva, and immunosuppression (e.g.,

hydroxychloroquine)

• For dry eyes

•

• Avoid dry environments (e.g., shield eyes from wind, keep air humid)

• Artificial tears
THANK YOU!