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2/2016 vol. 29
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REVIEW
IgG4 Related Disease A Challenge for Pneumonologist
Dimitrios Theofilos 1
,
 
Evangelia Chondrou 1
,
 
Charalampos Marketos 1
,
 
Danai Bisirtzoglou 1
,
 
Nikolaos Staikos 1
,
 
Athanasios Zetos 1
,
 
George Politis 1
 
 
 
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1
Pneumonology Department, “Saint Savvas” Anticancer Hospital, Athens, Hellas
 
 
Corresponding author
Dimitrios Theofilos   

Pneumonology Department, “Saint Savvas” Anticancer Hospital, 171 Alexandras Av., 11522 Athens, Greece
 
 
Pneumon 2016;29(2):133-141
 
Article (PDF)
References (67)
 
KEYWORDS
IgG4-related disease
 lung
 fibrosing mediastinitis
 storiform fibrosis
 
ABSTRACT
IgG4-related disease (IgG4-RD) includes a wide spectrum of inflammatory and fibrous procedures that affect a variety of issues and organs and are accompanied by elevated serum IgG4 levels. The clinical presentation is quite heterogeneous as almost every organ can be influenced. Clinical, laboratory and histopathological features and criteria must be taken into account and both malignancies (solid tumors and lymphomas) and benign disorders be excluded for the diagnosis to be established. Intrathoracic involvement in IgG4-RD varies and includes the lung parenchyma causing nodules, masses, ground-glass opacities, infiltrates resembling consolidation and thickened bronchovascular bundles, the central airways resulting in stenosis, obstruction and bronchiectasis as well as the pleura with effusion and nodular lesions and the mediastinum. Hilar and mediastinal lymphadenopathy are the most common intrathoracic manifestation while fibroid mediastinitis is much more rare. Corticosteroids are the cornerstone of therapy and most of cases present complete or partial response. However, rates of recurrence after treatment termination are high. In addition, patients may develop IgG4-related extrathoracic disease during the next months or even years after the initial diagnosis.
CONFLICTS OF INTEREST
No financial conflicts of interest.
 
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