%0 Journal Article
%T Pulmonary and Orbital<i> </i>Bifocal IgG4 Disease: Case Report and Literature Review
%A Abasse Moussa Ounteini
%A Mamane Daou
%A Bad&#233
%A Malam-Abdou
%A Brah Souleymane
%A Amadou Djibrilla Almoustapha
%A Yerima Salifou
%A Seraphin Adjoh Komi
%A Etienne Devin
%A Habeeb Mahmoud
%A Anas Mehdaoui
%J Open Journal of Blood Diseases
%P 59-64
%@ 2164-3199
%D 2024
%I Scientific Research Publishing
%R 10.4236/ojbd.2024.142007
%X <b>Introduction:</b> IgG4 disease is an orphan, fibro-inflammatory autoimmune disease of recent discovery whose thoracic involvement is rarer. We report a case of Pulmonary and orbital bifocal IgG4 disease with a review of the literature. <b>Observation:</b> This is a 71-year-old patient with a history of hypertension, dyslipidemia, smoking cessation at 15 PA, with progressive dyspnoea, weight loss of 4 kg with PS = 0 for 2 months. The thoracic CT scan revealed 3 pseudotumoral lung lesions of the LIG, LID and LM. The histology of the two CT-guided lung biopsies and the LIG wedge had objectified inflammatory lesions without signs of malignancy. The evolution was marked by the occurrence of a right orbital edema. The cerebral scanner found a voluminous right orbital inflammatory pseudotumor. Biopsy with histology found fibroinflammatory lesions with lymphoplasmacytic infiltrates and positive immunolabeling with anti-IgG4 antibodies. The PET scanner had objectified pulmonary and pleural parenchymal consolidations and moderately hypermetabolic mediastinal ADP with max SUV between 3 and 6. The patient was put on corticosteroid therapy with a favorable outcome. <b>Conclusion:</b> IgG4 disease is rare and difficult to diagnose despite well-defined and consensual diagnostic criteria and classification. The discovery of new biomarkers facilitates the diagnosis and monitoring of patients. Well-codified corticosteroid therapy is effective but possibility of recurrence. The current challenge remains the lack of data on the follow-up of these patients to assess the risk of neoplasia (lymphoma).
%K Orphan Disease
%K Thoracic IgG4 Disease
%K Autoimmunity
%U http://www.scirp.org/journal/PaperInformation.aspx?PaperID=133862