A 41-year-old man developed swelling of the cervical, axillary and inguinal LNs up to 15 mm in diameter. Laboratory examinations were white blood cell (WBC): 11,800/μL (0.3% eosinophils); hemoglobin:12.6 g/dl; C-reactive protein (CRP): 1.1 mg/dl; total protein (TP): 9.3 g/dl; albumin: 2.8 g/dl. He had elevated levels of serum IgG (3945 mg/dl, normal range: 861–1747), IgG4 (1340 mg/dl, normal range: 11–121 mg/dl), and IL-6 (11.5 pg/ml, normal range: ≤4.0 pg/ml). Histological examination of his left inguinal LN revealed prominent lymphoplasmacytic infiltration in the inter-follicular area, but eosinophilic infiltrations were not apparent (Fig. 1a and b).
Immunohistochemically, marked IgG4+ plasma cell infiltration was observed and his IgG4+/IgG+ plasma cell ratio was over 40% (Fig. 1c and d). Chest CT scan was done. We began treatment with oral prednisolone at 30 mg/day (0.5 mg/kg/day). This slightly improve his chest CT findings, but not other clinical and laboratory findings. He tapered off prednisolone over a 20-month period. Thereafter, he had no symptoms, but chest X-ray findings showed gradual progression.
Four years after tapered off prednisolone, he presented with a two-month history of gradually worsening dry cough. Chest CT findings showed progression of the previously observed findings. Laboratory findings were: WBC: 8400/μL (neutrophil: 73.2%, lymphocyte: 15.0%, eosinophil: 5.5%); hemoglobin:10.9 g/dl; CRP: 3.65 mg/dl; TP: 10.1 g/ dl; and albumin: 2.5 g/dl. Bronchoalveolar lavage fluid (BALF) revealed 30.0% eosinophils, 4.3% lymphocytes, 4.0% neutrophils, 61.3%, macrophages and a CD4+/CD8+ ratio of 0.42.
Histological examination of his surgical lung biopsy at right S8 showed dense lymphoplasmacytic accumulations, mainly in the alveolar area adjacent to the perilymphatic stromal area (Fig. 2 a, b). Some focal dense eosinophilic infiltrations and collagenous fibrosis were also noted (Fig. 2 c, d), but few IgG4+ plasma cells were observed. Serum level of IgG and IgG4 were increased (6858mg/dl and 3140 mg/dl, respectively). Rheumatoid factor was negative, but myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) was positive at 117 U/ml (normal range: < 4 pg/ml) and at 552 pg/ml (normal range < 450 pg/ml), respectively. Urinalysis showed no active sediment. He showed no cutaneous evidence of vasculitis or stigmata of collagen vascular disease.
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