Patterns of Pulmonary Function Test (PFT) Abnormalities in Sarcoidosis

Rasheed, Ameer; Vasudevan, Viswanath; Arjomand, Farhad

doi:10.1378/chest.1389741

Cited by 1 publication

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“…An increase in lymphocytes with an elevated CD4/CD8 ratio heralds a spontaneous resolution or a desirable course under therapy, but an increase in neutrophils is associated with progressive disease requiring therapy [88]. PFTs may reveal reduced diffusion capacity and a restrictive pattern with loss of vital capacity, but also obstructive changes [89].…”

Section: Autoimmune and Autoinflammatory Diseases Associated With Pulmonary Granulomatosis Sarcoidosismentioning

confidence: 99%

Pulmonary granulomatosis of genetic origin

et al. 2021

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Granulomatous inflammation of the lung can be a manifestation of different conditions and can be caused by endogenous inflammation or external triggers. A multitude of different genetic mutations can either predispose patients to infections with granuloma-forming pathogens or cause autoinflammatory disorders, both leading to the phenotype of pulmonary granulomatosis. Based on a detailed patient history, physical examination and a diagnostic approach including laboratory workup, pulmonary function tests (PFTs), computed tomography (CT) scans, bronchoscopy with bronchoalveolar lavage (BAL), lung biopsies and specialised microbiological and immunological diagnostics, a correct diagnosis of an underlying cause of pulmonary granulomatosis of genetic origin can be made and appropriate therapy can be initiated. Depending on the underlying disorder, treatment approaches can include antimicrobial therapy, immunosuppression and even haematopoietic stem cell transplantation (HSCT). Patients with immunodeficiencies and autoinflammatory conditions are at the highest risk of developing pulmonary granulomatosis of genetic origin. Here we provide a review on these disorders and discuss pathogenesis, clinical presentation, diagnostic approach and treatment.

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Section: Autoimmune and Autoinflammatory Diseases Associated With Pulmonary Granulomatosis Sarcoidosismentioning

confidence: 99%