Idiopathic Pulmonary Fibrosis from the Perspective of an Outpatient Pulmonologist
Idiopathic pulmonary fibrosis represents a rare disease of lung tissue with an as-yet-unexplained etiology. In the framework of differential diagnosis, it is necessary to consider the disease, as early diagnosis combined with the deployment of antifibrotic therapy extends patients' lives.
Introduction
Idiopathic pulmonary fibrosis is a rare progressive fibrotic disease of the lung tissue of unclear etiology characterized by progressive shortness of breath. The disease primarily affects adult patients. It occurs in individuals over the age of 50, with a median age at diagnosis between 60 and 70 years. Patients typically die from respiratory failure within 2 to 5 years after diagnosis. Early diagnosis combined with effective antifibrotic therapy increases survival time.
Diagnosis of the disease in the pulmonology clinic
The diagnosis of idiopathic pulmonary fibrosis is established based on clinical and imaging examinations after excluding other etiologies of the symptoms. Basic clinical symptoms include progressive effortful and later resting dyspnea, easy fatigue, cough, and in later stages, cyanosis due to hypoxemia. The disease progression is usually gradual, with worsening symptoms, but some patients experience acute exacerbations.
In clinical examination, 75% of patients show clubbing of the fingers with nails shaped like watch glasses, and auscultation reveals crackles over the lung bases. The disease affects only the lung tissue, without systemic symptoms.
The primary diagnostic method is high-resolution computed tomography (HRCT) of the lungs. Typical signs include reticulation and strip-like opacities with a honeycombed lung appearance, most evident at the lung bases and subpleurally. On the contrary, so-called active changes in the form of ground-glass opacities, infiltrates, and granulomas are absent (or minimal). On a plain posteroanterior chest X-ray, reticular shadows in the lower lung fields are characteristic of the disease, and in advanced cases, this finding is diffuse.
The clinical picture combined with HRCT examination forms the basis. Additional diagnostic tools include spirometry, which shows restrictive ventilatory impairment, the severity of which determines the administration of the antifibrotic of choice—pirfenidone. As part of the differential diagnosis, bronchoalveolar lavage should also be performed in patients suspected of having idiopathic pulmonary fibrosis; lung tissue biopsy is mainly reserved for unclear findings.
The potential use of an application in diagnostics
In the context of diagnosing idiopathic pulmonary fibrosis, the free online application IPFchecker can be utilized as a tool for the early detection of patients. The web application includes a calculator evaluating the possible presence of usual interstitial pneumonia based on basic clinical findings and HRCT criteria. The application's algorithm is based on the current Czech guidelines. Another feature of the application is the ability to consult HRCT findings with a radiologist from a specialized center. Thanks to the application, it is also possible to provide the patient with information that facilitates the path to further examinations.
Conclusion
Early diagnosis of the disease in a pulmonology clinic based on clinical and imaging examinations is a key prognostic factor. With an early diagnosis, antifibrotic therapy can be initiated, which slows disease progression and improves the patient's quality and lifespan.
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Sources:
1. Doubková M. Idiopathic pulmonary fibrosis—early diagnosis still matters. Medicine for Practice 2017; 14 (1): 45–48.
2. Vašáková M. Innovations in the treatment of idiopathic pulmonary fibrosis. Internal Medicine 2014; 16 (5): 189–191.
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