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04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Unusual presentation of progressive systemic sclerosis.
S U Chowdhury1, M A Miah, M I Mahmud
1Department of Dermatology and Venereology, Mymensingh Medical College & Hospital, Mymensingh, Bangladesh.
Mymensingh Medical Journal : MMJ
|July 16, 2008
Summary
This case study describes an atypical presentation of progressive systemic sclerosis (PSS) in a 38-year-old female. Despite negative serology, clinical and histopathological findings confirmed the diagnosis, highlighting diagnostic challenges in scleroderma.
Area of Science:
- Rheumatology
- Dermatology
- Internal Medicine
Background:
- Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease characterized by ত্বক thickening, fibrosis, and vascular abnormalities.
- Early diagnosis and management are crucial for improving patient outcomes and preventing irreversible organ damage.
Observation:
- A 38-year-old female presented with joint pain, skin tightness, dyspigmentation, hand deformities, and dysphagia.
- Clinical examination revealed hidebound skin, salt-and-pepper dyspigmentation, claw-like hands, and restricted chest movement.
- Histopathology confirmed scleroderma, while lung function tests indicated restrictive lung disease.
Findings:
- The patient was diagnosed with progressive systemic sclerosis (PSS) despite negative antinuclear antibody (ANA), rheumatoid factor (RA), and venereal disease research laboratory (VDRL) tests.
- Atypical features included the absence of Raynaud's phenomenon and digital ischemia.
- Barium swallow showed mild esophageal dilation, consistent with PSS involvement.
Implications:
- This case highlights the importance of considering PSS even with seronegative results and atypical clinical manifestations.
- Accurate diagnosis relies on a combination of clinical, histopathological, and radiological findings.
- Further research into seronegative PSS and its management strategies is warranted.
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