Case Report on Systemic Sclerosis with Interstitial Lung Disease

We are pleased to present an insightful case report authored by Dr Gangai Amaran , our esteemed Consultant Specialist in Pulmonology at Pun Hlaing Hospitals. The case report details the clinical presentation, diagnosis, and treatment of a 42-year-old female patient with systemic sclerosis complicated by interstitial lung disease (SSc-ILD).

Systemic Sclerosis with Interstitial Lung Disease: A Case Report

Abstract

Systemic sclerosis (SSc), also known as scleroderma, is a chronic autoimmune connective tissue disorder characterized by skin thickening and fibrosis. The disease frequently involves multiple organs, particularly the lungs, leading to interstitial lung disease (ILD). Early diagnosis of SSc-ILD is critical for preventing disease progression and improving outcomes. This case report details the clinical presentation, diagnosis, and treatment of a 42-year-old female patient with SSc-ILD, aiming to raise awareness of this rare condition and emphasize the need for early detection and effective management strategies.

Case Presentation

A 42-year-old female patient, with a history of hypertension, presented with a one-year history of shortness of breath, cough, wheezing, and desaturation (SpO2 at 84%), which acutely worsened over two days.

Clinical Examination

The patient's examination revealed bilateral basal coarse crackles and scattered wheezing. Key investigations included:

• Chest X-ray: Bilateral lower zone consolidation.
• Arterial Blood Gas (ABG): Type 1 respiratory failure.
• Spirometry: Severe restrictive lung disease.
• Electrocardiogram (ECG): Sinus tachycardia.

Treatment and Initial Management

The patient was admitted to the ICU and received high-flow oxygen therapy. She was administered intravenous hydrocortisone (100 mg q8 hourly) and empirical antibiotic therapy with combination of Ceftriaxone and?Sulbactam (1.5 mg IV Bd).

Laboratory Investigations

• White Blood Cell Count (WBC): 9100/mm3, with neutrophilia (81%) and lymphopenia (13.8%).
• Inflammatory Markers: C-reactive protein (CRP) - 73 mg/L, erythrocyte sedimentation rate (ESR) - 100 mm/hr, antistreptolysin O (ASO) - 215 IU/mL, procalcitonin - 0.07 ng/mL.
• Microbiology: Negative sputum acid-fast bacillus (AFB) and culture. COVID-19 rapid antigen test - Negative.

Diagnostic Imaging and Autoimmune Panel

• High-Resolution Computed Tomography (HRCT): Subpleural ground-glass opacities and scattered intra- and interlobular interstitial thickening with a crazy paving pattern, indicative of post-COVID sequelae organizing pneumonia (interstitial lung disease).
• Autoimmune Panel: Rheumatoid factor (RA factor) - Negative Anti-cyclic citrullinated peptide (Anti-CCP) - Negative Antinuclear antibody (ANA) - Positive ANA 23 panel: Scl-70 (+++) ANA indirect immunofluorescence: Strong positive (1:800 nucleolar pattern)

Final Diagnosis

Based on the clinical, radiological, and laboratory findings, the patient was diagnosed with systemic sclerosis with interstitial lung disease (SSc-ILD).

Treatment and Outcome

The patient received the following treatment regimen:

• Intravenous Cyclophosphamide
• Intravenous Methylprednisolone
• Intravenous Ifosfamide + Mesna
• Oral Nintedanib (100 mg)

The patient showed remarkable clinical improvement and was discharged without the need for oxygen support.

Discussion

Systemic sclerosis (SSc) is a rare and chronic autoimmune disease characterized by skin thickening and fibrosis, with variable clinical manifestations. Lung fibrosis occurs in approximately 80% of patients with SSc, with about 25% developing progressive ILD. SSc-ILD has a 10-year mortality rate of around 40%, making it a leading cause of morbidity and mortality in these patients. Early diagnosis is challenging due to non-specific symptoms such as cough, dyspnea, and chest pain. High-resolution computed tomography (HRCT) is essential for diagnosing and assessing SSc-ILD, along with a thorough clinical examination.

Conclusion

To our knowledge, this is one of the first detailed case reports describing the clinical presentation and HRCT findings of a patient with SSc-ILD in Pun Hlaing Hospitals group. This case underscores the importance of early detection, strict management, and the development of safe and effective treatments to improve outcomes and disease progression in patients with SSc-ILD.

Keywords: Systemic sclerosis, scleroderma, interstitial lung disease, case report, autoimmune disease, high-resolution computed tomography.

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