Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis

Abstract

Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. A 35-year-old female presented with exertional dyspnea, dry cough and weight loss. Physical examination demonstrated platythorax, suprasternal notch deepening and fine rales over the upper lobes. Blood count, serum biochemistry, autoimmunity and serologic markers for collagen vascular diseases were within normal limits. Arterial blood gases demonstrated a low pO2 (48 mm Hg) and a high pCO2 (54 mm Hg) values. Chest x-ray showed bilateral parenchymal fibrotic lesions, left pneumothorax, bronchiectasis in the middle and pleural thickening in the upper lung zones while HRCT revealed bilateral apical pleural thickening, traction bronchiectasis, subpleural reticulations, ground-glass opacities and honeycombing in the upper lobes. Bronchoscopy, BAL cytology, smear and culture did not reveal any pathologic findings. Relevant with the clinical, laboratory, radiologic manifestations and the differential diagnosis with other interstitial lung diseases, PPFE was the final diagnosis. The aim of this case report was to present the clinical manifestations of our case. The second crucial objective was to establish a diagnostic scoring system relevant with the literature and the clinical manifestations of the patient.

Diagnosis of pleuroparenchymal fibroelastosis: A review

“…The new assessment score analysis includes a much better descriptive and conclusive delineation for an unequivocal diagnosis compared with the assessment protocol that we had presented previously (36). The new protocol aims to maximize the possibility of diagnosis by inclusion of new criteria and excluding subjective inclusions such as patient symptoms that may arise in many other lung diseases.…”

Clinical Diagnosis of Pleuroparenchymal Fibroelastosis

“…Since this situation necessitates making the diagnosis according to clinical manifestations in the vast majority of patients, the diagnostic evaluation score we have introduced provides a great support to clinicians both for the definitive diagnosis of the disease and the differential diagnosis with other ILDs. We have reached an accurate defingtive diagnosis in two PPFE patients by using the diagnostic assessment score analysis (Tables 1 and 2) without the slightest reqiurement for tissue biopsy [35,36]. This analytical approach, besides its usefulness in terms of an accurate and definitive diagnosis, provides an effective approach to clinicians by creating a diagnostic pathway for the differential diagnosis with other interstitila lung diseases and for the evaluation of suspicious pleuroparenchymal fibroelastosis cases to preclude the redundant diagnostic interventions.…”

“…Platythorax is a frequent imaging manifestation that occurs due to extreme weight loss and decreased BMI of increased workload of respiration. Posterior tracheal border and spine convergence along with a notable deep suprasternal notch appearence may develop because of reduced upper thoracic volume and progressive weight loss that emerge as the other crucial diagnostic HRCT findings tissue biopsy evaluation [35] while a recent version of this algorithm comprising more objective diagnostic criteria is under publication. A new diagnostic algorithm, which includes more precise and objective diagnostic criteria for a definitive and accurate diagnosis of PPFE is still under publication [36].…”