RESUMO
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by significant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells within affected tissues, with or without elevated serum IgG4 levels. The prevalence of IgG4-RD remains largely undetermined due to diagnostic challenges, as the condition is frequently unrecognized or misdiagnosed. This report describes a case of a 63-year-old man who was ultimately diagnosed with this rare condition after an extensive two-year period of elusive symptoms. Initially presenting with intermittent body pains and fluctuating fever, his condition progressively evolved to include severe right orbital swelling with marked tenderness and ecchymosis, recurrent non-tender nodules on his arm, and diminished vision. A detailed review of his medical history prompted the consideration of IgG4-RD, leading to the measurement of serum human IgG4 levels, which were found to be significantly elevated at 1504 mg/L (normal range: 39.2-864 mg/L). Following his diagnosis, treatment with glucocorticoids (0.6 mg/kg for one month) was initiated, resulting in a positive clinical response. This case emphasizes the critical importance of considering less common conditions in the differential diagnosis of patients presenting with complex, multi-system symptoms.
RESUMO
Neurosarcoidosis is a rare manifestation of sarcoidosis, posing diagnostic challenges due to its varied clinical presentation and the lack of definitive diagnostic tests. We present a case of a 46-year-old African American female with progressive ascending bilateral sensory loss, weakness, and a bifrontal headache. Despite undergoing extensive diagnostic workup including cerebrospinal fluid analysis, neuroimaging, and bronchoscopic evaluation, a definitive diagnosis remained elusive. The patient underwent an open cervical spinal cord biopsy, which did not yield conclusive evidence of neurosarcoidosis. Subsequent complications included suspicion of an epidural abscess and post-operative cervical kyphosis. This case underscores the diagnostic dilemma and potential complications associated with the evaluation and management of neurosarcoidosis, highlighting the importance of a multidisciplinary approach in such cases.