Immunoglobulin G4–related Lung Disease: CT Findings with Pathologic Correlations

Abstract

IgG4-related lung disease manifested as four major categories of CT features. Pathologically, these features corresponded to IgG4-related sclerosing inflammation along the intrapulmonary connective tissue.

A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

“…Some patients present initially with respiratory symptoms, such as dry cough or dyspnea, whereas 75% of patients are asymptomatic and the disease is found incidentally by abnormal shadows on chest X-rays. Although IgG4-related PD is associated with a variety of radiologic abnormalities [49], diffuse lymphoplasmacytic infiltration has been observed in all lesions, with irregular fibrosis and obliterative vascular changes being more common in solid areas [48]. Hilar and pancreatic accumulation of gallium-67 has been reported as characteristic of the active stage of AIP when serum IgG4 concentrations are high [50].…”

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 II. Extrapancreatic lesions, differential diagnosis

“…Thoracic CT showed various lung lesions, bronchial wall thickening, nodules, interlobular thickening, infiltration in the middle and lower lung fields (Fig. 3a, b) [28], and honeycombing in the lower lung field [40]. While IgG4-related respiratory lesions of interstitial pneumonia, nodular lesions, localized ground glass-opacity (GGO), and pleural lesions sometimes occurred without a pancreatic lesion [16,[41][42][43][44][45], a definitive diagnosis of IgG4-related respiratory lesions was difficult in patients with intrathoracic lesions alone, as IgG4-bearing plasma cells have also been observed in other types of lung lesions [46].…”

Hemoptisis recurrente asociada a falla renal y hematuria: otra de las mil caras de la enfermedad relacionada a IgG4

“…Las manifestaciones clínicas varían desde lo asintomático hasta la tos, hemoptisis, disnea y dolor torácico 13,17 . Se han descrito cuatro patrones radiológicos de compromiso pulmonar: nódulos sólidos (el más frecuente), broncovascular (con engrosamiento de los haces broncovasculares y tabiques interlobulares), alveolo-intersticial (presencia de panal abeja, bronquiectasias, opacidad en vidrio esmerilado difusa) y opacidades en vidrio esmerilado "redondeadas" 14,18 . En muchos casos, el compromiso puede ser muy similar al de la sarcoidosis 19 .…”