Title: MORNING REPORT
1MORNING REPORT
2Sjogrens SyndromeChronic inflammatory
disorder featuring lymphocytic infiltration of
exocrine glandsCharacterized by dry eyes
(keratoconjunctivitis sicca) and dry mouth
(xerostomia)May be primary, or associated
with-Rheumatoid arthritis-Systemic Lupus
Erythematosus-Scleroderma
3Sjogrens diagnosismostly clinicaldry eyes,
dry mouthhelpful tests-tests to document
above(e.g. whole mouth sialometry)-autoantibodies
(anti-Ro/SSA anti-La/SSB)-salivary
histopathology showing foci of lymphocytes-tests
to rule out other connective tissue disorders
4Whole mouth sialometryComplicated process
whereby a patient spits into a cup for 15
minutes. Flow of less than 0.1 cc/min is
significant.
5Sjogrens can affect all organ systems How
often does it affect the lungs?
Answer Pretty often
620 Patients followed up to 60 months after
diagnosis of primary Sjogrens, 9 developed
pulmonary symptoms (restrictive PFTs and CXR
showing interstitial involvement)Azathioprine
had some benefit.Interstitial lung disease in
primary Sjogren's syndrome. Clinical-pathological
evaluation and response to treatment.Am J
Respir Crit Care Med. 1996 Sep154(3 Pt 1)794-9
7Of 36 Patients with known Sjogrens, 75 were
found to also have pulmonary involvement.
Respiratory manifestations in primary
Sjogren's syndrome. A clinical, functional, and
histologic study. Chest 1985 Aug88(2)226-9.
8Difference between secondary and primary
Sjogrens SyndromeLung involvement was more
frequent and severe in patients with the
secondary form of the syndromeMore severe
impairment of diffusion capacity in patients with
Raynaud's phenomenon. Lung involvement in
Sjogren's syndrome a comparison between patients
with primary and with secondary syndrome. Ann
Rheum Dis 1985 Jul44(7)455-61.
9Treatment(no control studies)Steroids are
mainstay 1 mg/kg for six months with PFTs and CT
at 2 and 6 months to follow for any
improvement.Azathioprine improved FVC at 6
monthsCyclophophamide and cyclosporine after
failure