IgG4-related systemic disease (IgG4-RSD) is an emerging autoimmune disorder that may affect several organs, with signs of organ fibrosis, storiform masses for hystopathological plasmacellular infiltration and plasmatic elevation of IgG4. This clinical condition frequently occurs in the sixth decade and may be considered an autoimmunity of the elderly; the disease may have a smouldering course with frequent misdiagnosis for the co-occurrence of comorbidity and clinical complexity.
The present case report describes the clinical case of an 81 years old woman admitted to the geriatric ward for remittent fever and functional decline. The past clinical history reported an isolated CT scan suggestive of retroperitoneal fibrosis of unknown origin with and a drug regimen that included chronic corticosteroids (prednisone 5 mg oad). The in hospital diagnostic workout demonstrated the presence of a thoracic aneurysm.
Several possible diagnoses among inflammatory, autoimmune (connective tissue disease, vasculitis, sarcoidosis, amyloidosis), infectious (mycotic) or neoplastic conditions were ruled out, as well as any drug association with higher risk of retroperitoneal fibrosis.
Thus, the clinical hypothesis of an IgG4 chronic periaortitis was formulated due to the co-occurrence of all the three major components: the presence of a retroperitoneal fibrosis, IgG4 related abdominal aortitis and peryaneurysmal fibrosis. Patient’s comorbidity did not allow performing the histological analysis. The present clinical case is original and adds knowledge to the 76 cases of thoracic aortitis due to IgG4 systemic disease out of the 3482 cases of disease reported so far. Further clinical investigation is needed to provide a homogeneous diagnostic workout for tailored early therapeutic intervention on the single geriatric patient. Moreover, a growing awareness of the disease is needed, especially in geriatrics, to providing a better standard of care and to improving the disease clinical knowledge and management.