Respiratory involvement in IgG4-related Mikulicz’s disease

Abstract

'Immunoglobulin G4 (IgG4)-related disease' is a new clinical concept of multi-organ diseases, with Mikulicz's disease (MD) being a clinical phenotype of IgG4-related disease. To clarify the clinical characteristics of respiratory involvement associated with IgG4-related MD, we retrospectively assessed 25 patients with MD, 11 (44%) of whom had allergic symptoms, and 7 (28%) of whom complained of respiratory problems. Thirteen patients (52%) presented with pulmonary and/or mediastinal lesions (P-MD) on chest computed tomography (CT), and 11 (44%) had lesions limited to the lacrimal and/or salivary glands (L-MD). Mean serum total protein, IgG, and IgG4 concentrations were significantly higher and CH50 was significantly lower in the P-MD than in the L-MD group. Immune complex was present only in the P-MD group. Chest CT images showed bronchial wall thickening, consolidation, nodule(s), interlobular thickening, ground glass opacity, pleural thickening/effusion, and mediastinal lymphadenopathy. Five of seven patients who underwent histological examination of the lungs had abundant IgG4-positive plasma cell infiltrates (IgG4/IgG-positive plasma cells >40%), but the other two did not. These findings suggest that respiratory lesions are not rare in patients with IgG4-related MD, and that they present with various manifestations. IgG4-related MD should be differentiated from similar diseases, such as sarcoidosis, bronchial asthma, Sjögren's syndrome, and malignant lymphoma.

Current concept and diagnosis of IgG4-related disease in the hepato-bilio-pancreatic system

“…IgG4-RD has been found to affect the pancreas [9, 10], bile duct [10, 11], lacrimal glands [10, 12], salivary glands [10, 12], central nervous system [10, 13, 14], thyroid [10, 15, 16], lungs [10, 17, 18], liver [10, 19, 20], gastrointestinal tract [10, 21–24], kidney [10, 25, 26], prostate [10, 27, 28], retroperitoneum [10, 29], arteries [10, 30], lymph nodes [10, 31], skin [10, 32], and breast [10, 33]. However, before the disease was identified, each organ lesion was described independently.…”

Clinical implications of serum IgG4levels in patients with IgG4-related ophthalmic disease

“…Not all IgG 4 -RD patients' serum IgG 4 levels are elevated,7 and up to 40% of patients with biopsy-proven IgG 4 -RD had normal serum IgG 4 levels 8. However, some reports showed that serum IgG 4 levels in patients with multiple organ involvement were higher,9 10 and correlated with disease activity 11. Amid the controversy in IgG 4 -RD, the clinical interpretation of serum IgG 4 concentrations in IgG 4 -ROD has not yet been evaluated.…”

A case of IgG4-related pulmonary disease with rapid improvement

“…IgG4-related pulmonary disease (IgG4-PD) often develops in males aged about 60 years. The frequency of allergic predisposition, such as rhinitis and bronchial asthma, in patients with IgG4-PD is higher than that in the general population [11]. A possible relationship between asbestos exposure and IgG4-PD as well as allergic condition has been proposed [12].…”

“…Additionally, hypocomplementemia, high serum IgE, and positivity for RF and ANA are often observed [11,13,14]. Various abnormal radiographic patterns, such as bronchial wall thickening, consolidation, nodule(s), ground-glass opacity, interlobular thickening, honeycombing, pleural thickening/effusion, and mediastinal lymphadenopathy, have been observed.…”

“…Corticosteroid therapy has been regarded as effective therapy for IgG4-RD. Most patients with IgG4-PD respond well to prednisolone at an initial dose of 20-50 mg, but some patients develop extra-pulmonary lesions [11] or experience recurrence of radiologic changes [15]. Further investigation is necessary to determine the optimal initial dose of corticosteroid and collect long-term follow-up data.…”