Idiopathic Pleuroparenchymal Fibroelastosis - A Rare Condition

Introduction

Pleuroparenchymal fibroelastosis, or PPFE, can develop without any known cause or underlying disease ("idiopathic PPFE") and with other diseases or conditions. According to the worldwide classification of idiopathic interstitial pneumonia (IIP), idiopathic PPFE has been included as one of the rare forms of idiopathic interstitial pneumonia. Upper-lobe-dominant progressive pulmonary fibrosis is one of the features of this condition. It also has a peculiar histology of lung swelling and collagenous inflammation. It occurs between the thickening and degeneration of elastic fibers, loss of elasticity in connective tissue, especially of the skin pleura and the body wall, and intra-alveolar cellular protein scarring. All of these features are found within the alveoli.

The clinical history of PPFE is comparable to that of other chronic fibrosis as the disease moves at a slow pace and progresses similarly.

What Are the Signs and Symptoms?

These are some of the symptoms of PPFE:

• A hacking cough.

• Shortness of breath, especially when exercising.

• Dull ache around the lungs.

• Dyspnea (a feeling of being unable to breathe thoroughly or quickly enough).

• Effortless cough.

• Loss of weight.

• Pneumothorax (accumulation of air or gas within the space separating the lungs and the thoracic wall, resulting in lung collapse).

• Chest discomfort.

• Chest wall malformation.

• Occasionally, audible crackles.

• Pneumothorax is triggered by air entering the chest cavity and collapsing the lungs.

What Are the Risk Factors?

Risk factors refer to the variables or conditions that increase the likelihood of a negative outcome or the occurrence of an adverse

• Transplantations involving the lungs, bone marrow, and hematopoietic cells.

• Chemotherapy.

• Occupational exposures, such as the inhalation of aluminosilicate dust particles.

• The oral history is related to frequent lower respiratory tract infections.

• The relationship between genetics and familial history is of significance.

What Is the Diagnostic Method?

1. Physical Examination

• Flattened Thoracic Cage - A flattened thoracic cage is another defining physical feature of PPFE patients. Physically, a flattened thoracic cage is the result of a congenital disposition or a secondary change of the thorax as a result of the progressive fibrosis of the upper lung lobes, which may inhibit swelling of the lungs and the constant blood flow in the upper lobes, resulting in an imbalance between ventilation and perfusion. Long-term follow-up from an initial phase of PPFE will clarify the significance of a thoracic cage that has been flattened.

• Clubbed Digit - The fingertips grow, and the nails become significantly curved from front to back. Rarely have clubbed digits been reported in PPFE patients.

• Late-Inspiratory Crackles - Late inspiratory crackles are caused by airway restriction. They are believed to result from the explosive opening of closed peripheral airways as movement increases during lung inflation, and critical pressure develops within lungs.

2. Respiratory Performance: PPFE is characterized by alveolar collapse with subpleural elastosis, intra-alveolar fibrosis, and pleural thickening, primarily in higher lobes. Total lung capacity (TLC) and forced vital capacity (FVC) decrease, whereas the ratio of forced expiratory volume in one second/forced vital capacity (FEV1/FVC) increases. These physiological alterations are identical to those associated with IPF. Fibrotic compression of the upper lobes causes compensatory overinflation of lower lobes, increasing the residual volume/total lung capacity (RV/TLC) ratio, a functional impairment not typically observed in IPF.

An impairment in gas exchange may also manifest as a restrictive impairment. The carbon monoxide diffusing capacity (DLco) decreases. When DLco is divided by alveolar volume (DLco/VA), the diffusing capacity is normal or marginally reduced in most cases. Although a rapid decline in FVC is associated with a poor prognosis in pulmonary fibrosis, the degree of annual decline may depend on the disease's stage at the outset of the annual change examination.

3. Imaging Techniques

• Chest X-Ray: A radiographic image of the chest region.

  • An anomalous constriction in the anterior to posterior dimension of the thorax resulted in a flattened thoracic cage.

  • There is a notable elevation in the levels of lung opacity due to inflammation.

  • Reticulated and nodular opacities in bilateral upper lung fields are observed.

  • In the advanced phase, fibrocystic opacities may be observed in the upper lung regions, while sporadic reticular opaqueness may be observed in the lower lung regions.

• CT Scan - A computed tomography (CT) scan of the chest region.

  • The initial phase, commonly referred to as "stage 1":

  • The apex region exhibits the presence of subpleural nodular in shape and reticular opacities, while the middle and lower lobes display relatively minor alterations.

In advanced level or stage.

• The appearance of fibrous opacities and tension due to airway obstruction that spreads to nearby lobes, as well as several fluid-filled sacs and large lesions (cysts) in the upper lung regions.

• In the lower lobes of both lungs, there are uneven cellular opacities between the pleura and the body wall. This pattern looks like normal interstitial pneumonia (UIP), which is the most common type of interstitial pneumonia.

What Are the Treatment Procedures?

• The progression of idiopathic PPFE is typically prolonged, and it is resistant to treatment with steroids or immunosuppressive drugs.

• There does not appear to be any effective treatment option for chronic fibrosing interstitial pneumonia.

• In the advanced stage, infection management is essential, and home oxygen therapy is required if the patient has hypoxemia.

• The fibrotic process in pulmonary fibrosis may be broken down into collagenosis(Any disease that specifically affects the connective tissues of the body.) and elastosis(A medical disorder characterized by the thickening and degradation of elastic fibers and the reduction of elasticity in connective tissue.) Collagenosis is the predominant process in PPFE.

What Is the Prognosis?

The progression of idiopathic PPFE is typically very slow, and it is resistant to treatment with steroids or immunosuppressive drugs. There does not appear to be any effective treatment option for chronic fibrosing interstitial pneumonia. In the advanced stage, infection management is essential, and home oxygen therapy is required if the patient has hypoxemia. This is also true for IPF. Infection with Aspergillus will be superimposed, particularly in the fibrocystic lesions of the disease, as seen in the advanced stage of idiopathic pulmonary fibrosis (IPF).

Elastosis is the predominant process in PPFE, however, both procedures are found in PPFE and IPF. The fibrotic process in pulmonary fibrosis may be broken down into collagenosis and elastosis, as collagenosis is the predominant process in PPFE.

Conclusion

PPFE is a rare disorder and Idiopathic PPFE is regarded as one of the rarest occurring fibrosis. Due to the rarity of fibrosis cases, its clinical aspects have not yet been thoroughly understood. The upper-lobe-predominant lesions and other symptoms are the typical signs of idiopathic PPFE. The natural history and prognosis of this illness, as well as the link between idiopathic and secondary PPFE, are all important factors to be considered. Lung transplantation is the only reported therapeutic treatment for PPFE. A comprehensive approach has resulted in a successful bilateral (involving both sides) lung transplant for PPFE.