Abstract
Systemic sclerosis (SSc) should be considered in all patients initially diagnosed
with idiopathic interstitial lung disease (ILD), even in the absence of classical
scleroderma cutaneous features. Systemic sclerosis sine scleroderma (ssSSc) is a rare
subtype of SSc, and the diagnosis requires the absence of characteristic skin thickening
but the presence of the three following criteria: (A) Raynaud's phenomenon or the
equivalent of abnormal nail fold capillaries, (B) positive antinuclear antibody (ANA),
typically with nucleolar or speckled immunofluorescence pattern, and (C) at least
one internal organ involvement of ILD, renal dysfunction, esophageal/bowel dysmotility
or pulmonary arterial hypertension; in the absence of an alternative rheumatological
diagnosis. The radiological and histopathological features of systemic sclerosis sine
scleroderma-associated interstitial lung disease (ssSSc-ILD) are commonly those of
non-specific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP)
that cannot help distinguish between idiopathic interstitial pneumonia, different
types of connective tissue diseases, or even different subsets of SSc. Therefore,
other than chest imaging, the use of nail fold capillaroscopy, positive serum ANA
antibody, echocardiogram, and esophagram are essential, in conjunction with the clinical
presentation for facilitating the diagnosis of ssSSc. We present a case of a 58-year-old
woman presenting with chronic dyspnea, a positive review of systems for Raynaud's
phenomenon, and found to have elevated nucleolar immunofluorescence pattern of ANA
with chest imaging consistent with the diagnosis of ssSSc-ILD. The uniqueness of this
case is that despite symptomatic alleviation with oral mycophenolate therapy, our
patient's restrictive lung disease on pulmonary function tests continued to decline,
requiring initiation of oral nintedanib therapy leading to stability and improvement.
However, due to the rarity of ssSSc, the use of oral nintedanib for systemic sclerosis-associated
ILD has only been formally assessed on patients with diffuse cutaneous systemic sclerosis
and limited cutaneous systemic sclerosis.
Key Indexing Terms
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Article info
Publication history
Published online: March 08, 2022
Accepted:
January 31,
2022
Received:
December 15,
2020
Identification
Copyright
© 2022 Published by Elsevier Inc. on behalf of Southern Society for Clinical Investigation.
