Hamman-Rich syndrome: a forgotten entity

Abstract

The following report outlines the case of a 76-year-old gentleman who presented to the hospital with acute interstitial pneumonitis, a rare and rapidly progressive type of idiopathic interstitial pneumonia. The patient initially presented with a three-week history of progressive shortness of breath and cough which was subsequently diagnosed as community acquired pneumonia. Treatment with oral antibiotics was unsuccessful resulting in re-presentation the following week with type one respiratory failure (hypoxemia without hypercapnia). Investigations revealed widespread inflammatory changes consistent with an acute inflammatory process -neutrophilia, elevated D-dimers, raised C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), as well as bilateral interstitial infiltrates on chest radiograph. Intravenous steroids, antibiotics and antiviral medications were initiated before an urgent transfer to the intensive care unit was required for intubation. An open lung biopsy, in conjunction with the clinical picture, confirmed the diagnosis of acute interstitial pneumonitis. The significance of this report is to highlight the rapid and destructive clinical course of a rare type of pneumonitis, which initially presented as a routine and innocuous diagnosis in our patient.

Old wine in new wineskins. Hamman-Rich syndrome has much in common with severe COVID-19: Do they share any link of pathogenesis or depend on the similar aetiological factor(s)?

“…The rapid progressing of respiratory failure is related to non-ventilatory (aka: "parenchymal") mechanism of decreased diffusion across alveoli-capillary barrier and altered perfusion of microcirculatory bed in lesser circulation. This acute syndrome affects apparently healthy individuals and is not associated with prior chronic lung diseases, smoking habits, age or gender [10]. At the same time the progression of the disease is not associated with comorbid background such as bronchial asthma and can affect people most often aged over 40, with a most typical age of 50-55 [11][12][13], although adolescent and pediatric cases were also described [14][15][16][17][18][19][20][21].…”

“…Some authors, stressing the importance of open thoracoscopic lung biopsy in diagnosis of Hamman-Rich syndrome, insist on single possible radical approach in its treatment -that is lung transplantation [10].…”

Viral Myocarditis in the Setting of Delayed Manifestation of Hamman-Rich Syndrome

“…This trait sets it apart from other similar disorders. Acute and chronic interstitial pneumonia differ in terms of pathologic lesions of varying ages and include interstitial inflammation in diverse lung locations, normal parenchyma, fibroblast foci, and honeycomb change [1]. Three phases of alveolar damage are associated with AIP: an initial exudative phase, a later organized proliferative phase, and a final fibrotic phase [2].…”