Pulmonary Hyalinizing Granuloma with Hydronephrosis

Abstract

A 49-year-old man was admitted for the evaluation of a bilateral mass shadow in his chest X-ray film. No definitive diagnosis was established either by brushing cytology or biopsy through bronchoscopy. No malignancies were suggested by general work-up. Both masses were surgically removed, and were diagnosed as pulmonary hyalinizing granuloma (PHG). Fifteen months later, low grade fever continued and the renal function decreased. Laboratory examinations revealed bilateral hydronephrosis with polyclonal hypergammaglobulinemia. The findings of abdominal CT and urography were compatible with retroperitoneal fibrosis. Steroid treatment completely reversed the initial abnormality in laboratory data and the symptoms disappeared.

Pulmonary Hyalinizing Granuloma With Associated Elevation in Serum and Tissue IgG4 Occurring in a Patient With a History of Sarcoidosis

“…4 Furthermore, there are a number of older reports in which PHG has been associated with lesions that have now been brought under the ISD umbrella, including sclerosing mediastinitis, retroperitoneal fibrosis, uveitis, and systemic idiopathic fibrosis. [5][6][7][8]22,23 Interestingly, our patient also had pleural fibrosis, which has been described as a thoracic manifestation of ISD. Our case is the first with a demonstration of increased serum and tissue IgG4 to specifically provide the link between PHG and ISD.…”

Pulmonary hyalinizing granuloma associated with Sjögren syndrome and ANCA MPO vasculitis

“…Imatinib mesylate, a tyrosine kinase (TK) inhibitor, is approved for the treatment of chronic myeloid leukaemia and FIP1L1/PDGFRA positive gene fusion hypereosinophilia. A potential role for imatinib in the treatment of idiopathic hypereosinophilic syndrome (HES) [3] and AAV has been recently proposed [4,5].…”

“…The etiology and pathogenesis remain unclear, but most authors have hypothesized that patients with PHG show evidence of underlying autoimmune phenomena [2][3][4][5]. Gorini et al [6] reported the first case of pulmonary hyalinizing granulomas in which the patient had antineutrophil cytoplasmic autoantibodies with a granular cytoplasmatic pattern; they suggest that PHG could be regarded as a localized form of Wegener's granulomatosis.…”

“…Although the association with autoimmune diseases is known [2][3][4]6,7] we provide the first description of a patient with PHG associated with pANCA vasculitis and Sjögren syndrome.…”

Non-diagnosed pulmonary hyalinizing granuloma (PHG) as a cause of sudden unexpected death

“…The PHG can be combined with other diseases, e.g. with retroperitoneal fibrosis, but a pathophysiologic relation between these diseases has not been proven [3,4].…”