Table 2 Seven cases of IgG4 related Tracheobronchial lesions

1 [11]

63/f

Cough

Mucosal edema and engorged vessels

1660

Submandibular gland neoplasm, autoimmune pancreatitis

oral prednisolone 1 mg*kg⁻¹*d⁻¹. All involvement improved

biopsy specimens from the bile duct showed infiltration of IgG4-positive plasma cells. Bronchial biopsy:diffuse inflammatory infiltrates consisting mainly of plasma cells, lymphocytes and scattered eosinophils with fibrosis with infiltration of several IgG4-positive plasma cells. The number of IgG4- positive cells was 30 per HPF。

2 [8]

22/f

Shortness of breath, wheezing, sore throat

mass surrounding larynx and upper trachea

N/A

none

Prednisolone and surgery

an IgG4-sclerosing pseudotumor, with fibrosis and a dense acute-on- chronic inflammatory infiltrate rich in plasma cells. This was associated with a proliferation of histiocytes and aggregates of lymphocytes. Immune-staining demonstrated mixed CD20+ B lymphocytes and CD3+ T lymphocytes. CD68 elucidated scattered histiocytes. The IgG/IgG4 plasma cell ratio was less than 50%.

3 [12]

70/M

None

Edematous and multiple central lesions and capillary dilatation in the primary bronchi

2600

Submaxillary gland and Parotid gland swelling, hypertrophic pachymeningitis

oral prednisolone

A lumbar puncture revealed pleocytosis (29.6/mm³: mononuclear,25.6/mm³, polymorphonuclear, 4/mm³) Biopsy specimens of the parotid gland and a bronchial elevated lesion: chronic inflammation and fibrosis in both lesions, as well as numerous plasma cell infiltrations. Immunohistochemical analysis indicated that the majority of plasma cells were IgG4+.

4 [7]

26/F

Shortness of breath and persistent dry cough

A tracheal lesion with 90% luminal obstruction

N/A

None

surgery

a dense lymphoplasmacytic infiltrate and fibrosis in a storiform pattern. The infiltrate was composed predominantly of lymphocytes and plasma cells, with interspersed fibroblasts and eosinophils. Immunostaining showed abundant IgG4-positive cells (155 per high-power field) and an IgG4/IgG ratio of approximately 0.9.

5 [9]

44/M

Sinus congestion, wheezing, dyspnea and cough

Inflammatory changes along the tracheobronchial tree.

2020

Pulmonary Parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

oral prednisone with 7.5 mg of maintenance therapy

Immunostaining for IgG4 highlighted 15 to 20 IgG4-positive plasma cells per high-power field. Outside submandibular gland pathology demonstrated numerous IgG4-positive plasma cells with an IgG4/IgG cell ratio of 0.6. Submandibular gland biopsy demonstrating the features of chronic sialadenitis. Increased immunoglobulin IgG4-positive plasma cells within the chronic inflammatory infiltrate in submandibular gland biopsy. Bronchoscopic biopsy demonstrating chronic inflammatory infiltrate and thickened basement membrane in bronchial mucosa. Increased IgG4-positive plasma cells within the inflammatory infiltrate in bronchial mucosa.

6 [10]

70/F

Dyspnea and facial edema

A smooth polypoid mass at the lower trachea.

N/A

Mass in the superior vena cava.

15 years ago: a mediastinal mass in the intratracheal and right lower paratracheal area

surgery

a mediastinal mass 15 years ago: diffusely fibrosclerotic change with proliferation of the fibroblasts and infiltration of chronic inflammatory cells.

mass in the superior vena cava:markedly increased lymphoid follicles, fibrosclerotic change of the stroma and a heavy infiltration of the plasma cells. In addition, immunohistochemical staining for IgG4 antibody demonstrated diffusion with strong positivity at the increased plasma cells

7 [13]

50/M

Chronic cough

Marked edema of the bronchial mucosa.

1180

Autoimmune pancreatitis, sinus mucosa thickening

Inhaled corticosteroids with systemic corticosteroid therapy

A bronchial biopsy specimen showed inflammation with marked infiltration of IgG4-positive plasma cells and storiform fibrosis.