Rare myopathy associated to MGUS, causing heart failure and responding to chemotherapy

Abstract

ralde, et al.. Rare myopathy associated to MGUS, causing heart failure and responding to chemotherapy.

Chemotherapy with stem cell transplantation is more effective than immunotherapy in sporadic late onset nemaline myopathy with monoclonal gammopathy

“…These new data are of a particular importance for patients with comorbidities who are not eligible for ASCT. As recently reported by Belhomme et al [ 9 ], a patient with a severe left ventricular dysfunction possibly due to MGUS related myopathy showed a significant improvement after treatment with four cycles of cyclophosphamide, bortezomib, dexamethasone without consolidation with ASCT.…”

Neuropathology in Pitié‐Salpêtrière hospital: Past, present and prospect

“…This kind of myositis is observed in patients treated with immune checkpoint inhibitors, used against cancer to improve the T cell response . Muscle biopsies also identified late‐onset sporadic nemaline myopathy, found in patients with monoclonal gammapathies (MGUS) …”

Impact of hematologic complete response in the treatment of sporadic late-onset nemaline myopathy associated with monoclonal gammopathy

“…Monoclonal gammopathy of undetermined significance (MGUS) may be associated with pathologies with severe neuromuscular manifestations, namely POEMS Syndrome and AL Amyloidosis [1][2][3][4]. Another lessknown association is with a rare disorder named sporadic late-onset nemaline myopathy (SLONM) [5][6][7][8][9][10] and happens in more than half of these patients [11,12].…”

“…Head drop, neck muscle weakness, or neck muscle pain are features described in more than half of the patients with SLONM associated with MGUS (SLONM-MGUS) [13]. Cardiac involvement is less common, but there are echocardiographic and electric conduction cardiac abnormalities in some patients [3,[11][12][13][14][15][16]. The first cases published suggested an autoimmune etiology, and it was uncertain whether monoclonal gammopathy was associated or coincidental [6].…”

“…Treatment of first cases using immunosuppressive strategies showed better results with intravenous immunoglobulin [11,17], mostly on non-MGUS associated cases [12]. Nonetheless, other immunosuppressive treatments (prednisone, cyclophosphamide, rituximab) or even plasmapheresis showed disappointing and inconstant outcomes which led the autoimmune etiology to be questioned, at least for some patients [3,5,6,10,14,18,19]. The hypothesis that SLONM-MGUS is a continuum of light-chain deposition disease (LCDD) is corroborated by the presence of light chain deposition in skeletal muscles observed in some cases described [20].…”