Pulmonary amyloidosis and unusual lung involvement in SLE

Abstract

The association of systemic lupus erythematosus (SLE) with amyloidosis is exceptional. We present a 37-year-old patient who was diagnosed five months earlier for SLE. She developed an acute episode of chest pain, cough and dyspnoea. Hypoxemia and obstructive changes in respiratory tests were present. The chest X-ray was repeatedly normal. Open lung biopsy revealed lupus pneumonitis with positive stain for immunoglobulins and complement, bronchiolitis obliterans, and pulmonary amyloidosis.

Rapidly Progressed Secondary Amyloidosis in a Patient with Mixed Connective Tissue Disease

“…At the moment, this is the only case, to our knowledge, who was examined for the allelic variants of the SAA 1 gene other than RA. Although several reports described cases with rapidly progressive SA in the short periods of time in rheumatic diseases such as SLE (15,16), vasculitis syndrome (17, 18) and Behçet's disease (19,20), allelic variants of the SAA 1 gene were not pursued. In general, it takes long time, often more than 15 years, to develop SA in RA patients (11,21,22 We speculate that inadequate control of inflammation of MCTD might have caused SA in our case.…”

Systemic amyloidosis and sacroiliitis in a patient with systemic lupus erythematosus

“…In this patient there was no chronic infectious or inflammatory disease other than SLE. In the literature only a few cases on the development of AA type amyloidosis in SLE have been reported [1][2][3][4][5][6][7][8][9][10]. In most of the reported cases, the patient had suffered from long-standing SLE [1][2][3]9].…”

“…In all reported patients with SLE, the most frequently involved organ was the kidney [1-3, 6, 7, 10]. Renal with pulmonary [6], pulmonary alone [4,5], gastrointestinal [7,8] and hepatic involvement [9] have also been described. Postmortem examinations have revealed the deposition of amyloid in the heart, spleen [1], liver, skin and other connective tissues [7].…”

“…Ter Borg et al reported immune deposits in the mesangium and the glomerular basement membrane in a SLE patient with secondary amyloidosis [3]. Marenco et al reported that open lung biopsy revealed lupus pneumonitis with positive staining for immunoglobulins and complement and pulmonary amyloidosis [4].…”

“…Systemic secondary (reactive) amyloidosis (AA) is mainly encountered as a complication of chronic rheumatic diseases, particularly rheumatoid arthritis, juvenile chronic arthritis and ankylosing spondylitis, but has been rarely reported in SLE [1][2][3][4][5][6][7][8][9][10]. Sacroiliitis is the characteristic feature of patients with seronegative spondyloarthropathy.…”