Background

Amyloidosis is a rare disease characterized by extracellular deposition of misfolded proteins, leadings to organ dysfunction, and can be localized or systemic. Localized amyloidosis describes deposits of local amyloid in tissues such as lungs, urogenital systems, or skin; these deposits are derived from monoclonal light chains without evidence of an underlying systemic clonal plasma cell disorder. Patients with localized amyloidosis do not develop systemic disease such as cardiac, renal, hepatic, or nerve involvement. Typically, localized amyloidosis is managed with surgical resection, while systemic disease is treated with combination chemotherapy and immunotherapy. This case describes symptomatic localized pulmonary amyloidosis, contributing to the limited data on its clinical manifestations and course.

Clinical Course

A 53 year old female with Sjogren’s and arthralgias presented for evaluation of pulmonary nodules. Biopsy showed amyloidosis; there was no evidence of systemic amyloidosis including negative abdominal fat biopsy and no evidence of clonal plasma cells in her bone marrow aspiration and biopsy; so, she was diagnosed with localized amyloidosis; however, the patient had multiple nodules, and resection was not an option

Her PET scan showed a mildly FDG avid 1.4 cm right lower lobe nodule and additional nodules with minimal FDG activity in the right middle and upper lobes, left upper lobe, and lingula

The patient was followed closely until and she began experiencing symptoms including chest pain and shortness of breath (SOB); so, we started combination therapy commonly used for systemic amyloidosis: Daratumumab, Bortezomib (Velcade), Cyclophosphamide, and Dexamethasone (Dara-VCD). She tolerated the treatment with symptoms resolving, with plans to complete six cycles and repeat CT Chest in 6 months.

Conclusion

This report documents a case of localized amyloidosis requiring systemic therapy (Dara-VCD). It suggests that systemic therapy may be utilized for patients with localized amyloidosis in symptomatic patients, for whom resection is not an option.