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2015 ; 9
(ä): 82
Nephropedia Template TP
gab.com Text
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English Wikipedia
Mechanic s hands in a woman with undifferentiated connective tissue disease and
interstitial lung disease--anti-PL7 positive antisynthetase syndrome: a case
report
#MMPMID25888844
De Langhe E
; Lenaerts J
; Bossuyt X
; Westhovens R
; Wuyts WA
J Med Case Rep
2015[Apr]; 9
(ä): 82
PMID25888844
show ga
INTRODUCTION: Interstitial lung disease can be idiopathic or occur in the setting
of connective tissue diseases. In the latter case it requires a different
treatment approach with a better prognosis. Interstitial lung disease can precede
the onset of typical connective tissue disease features by many years, and
therefore meticulous multidisciplinary follow-up is crucial. This case highlights
the diagnostic challenge and the need for intensified attention for subtle
clinical features when faced with interstitial lung disease in patients with
characteristics of a hitherto undifferentiated connective tissue disease. CASE
PRESENTATION: A 44-year-old Caucasian woman presented to our pulmonology
department with dyspnea, Raynaud's phenomenon and subtle swelling of fingers and
eyelids. Laboratory analysis and autoantibody screening was negative. She was
diagnosed with nonspecific interstitial pneumonia with a concurring
undifferentiated connective tissue disease. After four years of stable disease,
she presented with rapid pulmonary deterioration, myalgia, periorbital edema,
arthritis and a cracked appearance of the radial sides of the fingers of both her
hands. This clinical sign was recognized as mechanic's hands and a specific
search for the presence of antisynthetase antibodies was performed. She was found
to harbor anti-threonyl-tRNA synthetase antibodies. A diagnosis of antisynthetase
syndrome was made and she was treated with glucocorticoids and
immunosuppressives. CONCLUSIONS: This case highlights the difficulty in
fine-tuning the diagnosis when confronted with a patient with interstitial lung
disease and the suspicion of an underlying, yet undifferentiated connective
tissue disease. There is a strong need for clinical multidisciplinary follow-up
of these patients, with a high level of alertness to rare and specific clinical
signs. The diagnosis of the underlying connective tissue disease profoundly
influences the management of the interstitial lung disease. Recent data stress
that identification of the autoantibody specificity allows for further prognostic
stratification and therefore should be pursued.