There are many causes of distal RTA, which causes may be primary or (more commonly) secondary (due to paraproteinemia
, medullary sponge kidney, nephrocalcinosis, obstructive uropathy, or autoimmune disease, etc.
Erythema elevatum diutinum may be associated with autoimmune diseases such as Celiac disease, Crohn's disease and rheumatoid arthritis, as well as hematological disorders such as myelodysplasia, multiple myeloma, immunoglobulin A paraproteinemia
, and lymphoma.
Scleromyxedema is a generalized papular eruption with systemic implications characterized by sclerosis, paraproteinemia
, and mucin deposition in the dermis.
However, distinction should be made for patients with "secondary" bone loss such as hyperparathyroidism, hyperthyroidism, vitamin D deficiency, and paraproteinemia
, as BTMs may have utility in these higher-risk patient subsets.
In classic PG, usually occurring on the legs, autoimmune diseases such as inflammatory bowel disease, rheumatoid arthritis or another inflammatory arthritis, and paraproteinemia
may be seen.
Other common causes are vitamin deficiencies (vitamin B1, B6, B12), folate deficiency, paraproteinemia
, and hypo/hyperthyroidism.
The relationship between paraproteinemia
and ACP dysregulation is unclear.
While investigating for paraproteinemia
, type of paraprotein should always be mentioned, as different flow cytometric immunophenotyping marker studies are required to be carried out.
Serum and urine protein electrophoresis did not reveal paraproteinemia
, and quantitative serum immunoglobulin and serum free light chain (FLC) assays did not reveal light chain restriction or excess.
Both neoplastic and non-neoplastic disorders are associated with monoclonal paraproteinemia
HIV-associated nephropathy (HIVAN) Membranoproliferative glomerulonephritis Mesangial glomerulonephritis with immune deposits Minimal change disease Lupus-like glomerulonephritis Crystal-induced nephropathy Fanconi syndrome Allergic interstitial nephritis Thrombotic thrombocytopenic purpura/Hemolytic uremic syndrome Paraproteinemia
Lymphoma Table 2.
Trastornos relacionados con paraproteinemia
IgM: existe proteina monoclonal IgM, con sintomas derivados de la proteina monoclonal IgM, pero sin evidencias de linfoma o infiltracion medular.