CASE PRESENTATIONS
CASE 1
A 58-year-old Japanese man with a previous history of hypopharyngeal cancer visited Toyama Prefectural Central Hospital due to cough and chest tightness aggravated over four months. The patient was a current smoker and worked as a cook. No problems were noted upon auscultation. Blood test results showed an eosinophil count of 53 cells/μL, a C-reactive protein level of 10.09 mg/dL (normal range: 0 – 0.14), and an immunoglobulin G4 (IgG4) concentration of 131 mg/dL (normal range: 5 – 117) (Table 1). Chest CT revealed moderate diffuse wall thickening of the trachea and main bronchi (Figure 1A). Flexible bronchoscopy detected an imbricate, oedematous tracheal and bronchial wall (Figure 1B). Bronchial biopsy specimens indicated airway inflammation with moderate eosinophilic and mild plasmacytic infiltration (Figure 1C, Supplement 1).
Prednisolone 0.5 mg/kg/day was administered; the patient’s symptoms improved promptly, and the airway wall thickness exhibited a gradual reduction on chest CT after four months of treatment. The prednisolone dose was gradually reduced and stopped after one year of treatment.
CASE 2
A 46-year-old Japanese man visited Toyama Prefectural Central Hospital due to a cough that had persisted for one month. He was a current smoker. No problems were noted upon auscultation. Blood test results showed a C-reactive protein level of 6.58 mg/dL and an IgG4 concentration of 191 mg/dL (Table 2). Diffuse wall thickening of the trachea and main bronchi was observed on chest CT (Figure 2A), and flexible bronchoscopy showed an oedematous tracheal and bronchial wall (Figure 2B). Airway inflammation with mild plasmacytic infiltration was detected based on bronchial biopsy specimens (Figure 2C, Supplement 2). Most immunoglobulin G-positive plasmacytes were positive for IgG4, and the IgG4/CD138 ratio was 0.56.
After five days, the patient developed severe cryptogenic haemoptysis. We treated him with methylprednisolone 1 g for three days, after which prednisolone 0.5 mg/kg/day was administered. The patient’s symptoms disappeared promptly, and the airway wall thickness exhibited a gradual reduction on chest CT. The prednisolone dose was gradually reduced to 10 mg/day and was continued as a maintenance therapy to control his symptoms and inflammation.
CASE 3
A 76-year-old Japanese man with a history of bronchial asthma and chronic obstructive pulmonary disease for two years visited Toyama Prefectural Central Hospital due to a cough and fever of approximately 38℃. No problems were noted upon auscultation. Blood test results showed an eosinophil count of 518 cells/μL, a C-reactive protein level of 4.75 mg/dL, an IgG4 concentration of 51 mg/dL, and normal levels of myeloperoxidase and proteinase 3-anti-neutrophil cytoplasmic antibodies (Table 3). Chest CT showed diffuse wall thickening of the trachea and main bronchi (Figure 3A), and flexible bronchoscopy revealed an oedematous tracheal and bronchial wall (Figure 3B). The bronchial biopsy specimens indicated tracheitis with palisading granuloma and multinucleated giant cells in the subepithelial area of the tracheal mucosa (Figure 3C, Supplement 3).
We treated the patient with prednisolone 0.5 mg/kg/day. His symptoms disappeared promptly, and the airway wall thickness exhibited a gradual reduction on chest CT. The prednisolone dose was gradually reduced for six months and then stopped.