Systemic Lupus Erythematosus and Amyloidosis
Systemic lupus erythematosus (SLE) can rarely be associated with AA amyloidosis, but there is no established connection between SLE and amyloid sarcoidosis specifically.
Relationship Between SLE and Amyloidosis
- AA amyloidosis is a rare complication of SLE that has been documented in only a small number of case reports 1, 2, 3, 4
- AA amyloidosis in SLE typically occurs secondary to long-standing inflammation, which can lead to the deposition of amyloid A protein in various organs 1
- When amyloidosis occurs in SLE patients, it most commonly manifests as renal involvement with proteinuria, which should be considered in the differential diagnosis of proteinuria in SLE patients 1
- Systemic inflammation may predispose to the development of amyloidosis, requiring monitoring through proteinuria and microalbuminuria testing 5
SLE and Sarcoidosis Connection
- The coexistence of sarcoidosis and SLE in the same patient has been uncommonly reported in the literature 6
- A literature review identified only 25 published cases of concurrent SLE and sarcoidosis, suggesting this combination is rare but possibly underreported 6
- When both conditions coexist, sarcoidosis typically presents with mild pulmonary symptoms while SLE manifestations are highly variable 6
- There is no established connection between SLE and amyloid sarcoidosis specifically in the medical literature 6
Clinical Implications and Monitoring
- SLE patients should be monitored for systemic inflammation using markers including peripheral neutrophilia, CRP, and ESR 5, 7
- Patients with long-standing SLE should be evaluated for potential development of amyloidosis through regular monitoring of proteinuria and microalbuminuria 5
- In SLE patients with unexplained proteinuria, amyloidosis should be considered in the differential diagnosis 1
- SLE patients with cutaneous manifestations, cytopenia, renal involvement, and/or positivity for ANA and anti-dsDNA antibodies who develop pulmonary symptoms should be evaluated for potential concurrent sarcoidosis 6
Treatment Considerations
- When SLE and sarcoidosis coexist, treatment typically includes oral glucocorticoids, hydroxychloroquine, and conventional immunosuppressive drugs 6
- In severe cases of concurrent SLE and sarcoidosis, cyclophosphamide may be considered 6
- Controlling systemic inflammation in SLE is crucial to potentially prevent the development of secondary amyloidosis 1, 4
- Regular monitoring of organ function is essential in SLE patients, particularly those with long-standing disease, to detect complications such as amyloidosis early 7
Diagnostic Approach
- Histological examination with Congo red staining is necessary to confirm amyloidosis in SLE patients 1, 2
- Permanganate sensitivity testing can help identify amyloid A protein in tissue samples 1
- Elevated serum amyloid A protein levels may be present in SLE patients who develop AA amyloidosis 1
- For suspected sarcoidosis in SLE patients, histological evidence of non-caseating granulomas is required for diagnosis 6