Abstract
Undifferentiated connective tissue diseases (UCTDs) are clinical entities characterised by signs and symptoms suggestive of a systemic autoimmune disease, which do not fulfil the diagnostic criteria for a defined connective tissue disease. Lung involvement can complicate the course and management of the disease, often determining a worse outcome. Respiratory dysfunction as the first clinical manifestation has seldom been reported.We describe a case of a female patient who developed significant respiratory dysfunction as the principal clinical sign. Video-assisted thoracoscopy was performed and a histological pattern of nonspecific interstitial pneumonia (NSIP) was found. A pathological diagnosis suggested careful follow-up with extensive immunological screening which then detected Raynaud's phenomenon and positivity of antinuclear antibodies. After a multidisciplinary discussion (pneumologist, radiologist, pathologist and rheumatologist) a final diagnosis of NSIP associated with UCTD was made. The diagnosis of UCTD should be considered when NSIP is diagnosed even in cases with evident first clinical manifestations of severe respiratory dysfunction. A multidisciplinary approach in the field of interstitial lung disease with NSIP, also including rheumatologic expertise, is fundamental to achieve a prompt and correct diagnosis.
Highlights
The term undifferentiated connective tissue disease (UCTD) is used to define clinical entities characterised by features suggestive of CTD which do not meet the classification criteria of the American College of Rheumatology for a specific single disease, such as systemic lupus erythematosus, systemic sclerosis, polymyositis/ dermatomyositis, and Sjögren’s syndrome [1,2,3,4,5,6,7,8,9]
It may be difficult to distinguish these conditions from early phases of defined diseases such as systemic lupus erythematosus, systemic sclerosis and others
The natural history of these rare entities is variable: a high percentage of patients with UCTD maintain an undifferentiated clinical course and do not evolve to a distinct CTD, whereas some patients can evolve over time [4,7,8,11]
Summary
The term undifferentiated connective tissue disease (UCTD) is used to define clinical entities characterised by features suggestive of CTD which do not meet the classification criteria of the American College of Rheumatology for a specific single disease, such as systemic lupus erythematosus, systemic sclerosis, polymyositis/ dermatomyositis, and Sjögren’s syndrome [1,2,3,4,5,6,7,8,9]. Lung involvement as the first clinical manifestation of UCTD is rarely reported. Open lung biopsy was performed and based on a histological diagnosis of non specific interstitial pneumonia (NSIP) an accurate immunological follow-up was required.
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