BACKGROUND: We report a case of light chain proximal tubulopathy associated with lupus nephritis in a patient known to have systemic lupus erythematosus. The kidney can be injured in several ways in any of these disorders. Light chain proximal tubulopathy is a rare form of renal tubular injury that may occur in and complicate plasma cell dyscrasia, characterized by cytoplasmic inclusions of the monoclonal light chain within proximal tubular cells. Lupus nephritis is a common form of renal injury as it occurs in about 25-50% of adult patients with systemic lupus erythematosus.
METHODS: We present a 57-year-old African patient known to have systemic lupus erythematosus and hypertension presented with a new complaint of microscopic hematuria. A renal biopsy was performed and revealed lupus nephritis class II concurrently associated with light chain induced proximal tubulopathy. A subsequent bone marrow biopsy was performed, which revealed multiple myeloma.
CONCLUSIONS: We report a case of coincidental lupus nephritis and proximal tubulopathy featuring a combined constellation of rare histopathological features that might add to the relationship between systemic lupus and paraproteinemia.

Scleromyxedema is a rare and progressive disease that currently has no standard treatment. Triplet therapy with lenalidomide, bortezomib, and dexamethasone can be an effective therapy for scleromyxedema, especially in patients with refractory or relapsed disease.

There have been significant advances in the treatment of multiple myeloma in the last 2 decades. Approximately 25% of patients with newly diagnosed myeloma have some degree of kidney impairment. During the course of illness, nearly 50% of myeloma patients will develop kidney disease. Moreover, approximately 10% of myeloma patients have advanced kidney disease requiring dialysis at presentation. Hemodialysis is associated with a significantly reduced overall survival. In the setting of prolonged long-term overall survival due to the use of newer immunotherapeutic agents in the treatment of myeloma, patients with myeloma and advanced kidney disease may benefit from more aggressive management with kidney transplantation. Unfortunately, most data regarding outcomes of kidney transplantation in patients with myeloma come from single center case series. With the advent of novel treatment choices, it remains unclear if outcomes of kidney transplant recipients with myeloma have improved in recent years. In this descriptive systematic review, we coalesced published patient data over the last 20 years to help inform clinicians and patients on expected hematologic and kidney transplant outcomes in this complex population. We further discuss the future of kidney transplantation in patients with paraproteinemia.

暂无摘要。

Scleromyxedema (SM) is a sclerotic variant of lichen or papular mucinosis in which lichenoid papules and scleroderma-like features are both present. It is a rare deposition disorder characterized by generalized papular and sclerodermoid eruptions, mucin deposition, increased fibroblast proliferation, fibrosis, and monoclonal gammopathy (also known as paraproteinemia) mainly of the immunoglobulin G-lambda type in the absence of thyroid disease. It usually affects middle-aged adults and shows no gender or racial predilection. In addition to the skin findings and paraproteinemia, patients with SM have variable multisystem affections that mimic systemic sclerosis; the systems which are commonly involved include the gastrointestinal tract, musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems, leading to significant morbidity and mortality. Prominent symptoms include dysphagia, proximal muscle weakness, and dyspnea on exertion; less common but important findings include central nervous system involvement in the form of encephalopathy, convulsions, coma, and psychosis.

Paraproteinemia potentially causes peripheral neuropathy via an unknown underlying pathogenetic mechanism. We report a case of pathologically proven amyloid neuropathy with AL amyloidosis with an IgA kappa light chain, which was initially diagnosed as neuropathy associated with monoclonal gammopathy of undetermined significance. This case indicates that in cases of neuropathy with paraproteinemia, the other potential causes should be excluded by appropriate means, especially pathological evaluations.