7539 - Renal amyloid disease

DBQ: Link to Index of DBQ/Exams by Disability for DC 7539

Definition

Amyloid disease refers to a metabolic disorder marked by deposits of protein-polysaccharide complexes in organs and tissue. In this case, the disease occurs in the kidney.

Etiology

Although the cause of amyloid disease is unknown, it is thought to be associated with certain chronic pathological states including tuberculosis; bronchiectasis; rheumatoid arthritis; osteomyelitis; leprosy; Hodgkin's disease; syphilis; familial Mediterranean fever; and multiple myeloma. It may also have familial and hereditary aspects, and occur with aging.

Signs & Symptoms

The kidney may be enlarged, firm, and smooth. The person's face may be pale with waxy skin. Diarrhea may be present, and the urine may contain waxy casts, albumin, and hyaline. Nephrotic syndrome may be an early indication of the disease. There may be mild proteinuria with possible progression to massive proteinuria, and generalized edema.

Tests

Biopsy and microscopic examination with the use of dyes for diagnosis.

Treatment

Treatment of the condition may revolve around controlling the underlying disease, and it may include: monitoring intake and output, and electrolyte levels; hemodialysis; kidney transplantation; or surgical removal of localized amyloid tumors may be other courses of treatment. In addition, diet management, as well as medication for pain, may be included to decrease amyloid deposits and to control bowel complications.

Residuals

The disease may affect multiple organs and have widespread vascular involvement. There is potential for renal failure and for amyloid deposits to occur in the long bones and joints of patients on long-term hemodialysis. In the event of a kidney transplant, rejection is a lifelong major concern that requires antirejection medications for life. Health may continue to decline, although patients may improve with supportive therapies. There is potential for this disease to recur in a donor kidney.

Special Considerations

  • This disease, (condition), only in its active form , has a 3 year presumptive period and shall be granted service connection although not otherwise established as incurred in or aggravated by service if manifested to a compensable degree within the applicable time limits under 38 CFR 3.307 following service in a period of war or following peacetime service on or after January 1, 1947, provided the rebuttable presumption provisions of 38 CFR 3.307 are also satisfied. [38 CFR 3.309 [Disease subject to presumptive service connection], 38 CFR 3.309(a). [chronic disease].

  • May be entitled to special monthly compensation where the Veteran has a single service-connected disability rated as 100% with additional service-connected disability or disabilities independently ratable at 60% or more, which are separate and distinct from the 100% service-connected disability and involves different anatomical segments or bodily systems. See  38 CFR 3.350(i)(1) – Special Monthly Compensation (SMC).

  • The new criteria for the Genitourinary Disease and Conditions body system became effective November 14, 2021.  Review both current and historic criteria prior to any reduction for disabilities established before that date.  38 CFR 3.951(a) 

  • A report of the Institute of Medicine of the National Academy of Sciences has identified some long-term health effects, listed in the table embedded within 38 CFR 3.317(d), that potentially are associated with the infectious diseases listed in 38 CFR 3.317(c)(2). These health effects and diseases are listed alphabetically and are not categorized by the level of association stated in the National Academy of Sciences report. If a veteran who has or had an infectious disease identified in column A also has a condition identified in column B as potentially related to that infectious disease, VA must determine, based on the evidence in each case, whether the column B condition was caused by the infectious disease for purposes of paying disability compensation. This does not preclude a finding that other manifestations of disability or secondary conditions were caused by an infectious disease. See 38 CFR 3.317(d) - Long-term health effects potentially associated with infectious diseases. 

  • If a veteran presumed service connected for one of the diseases listed in 38 CFR 3.317(c)(2) is diagnosed with one of the diseases listed in column “B” in the table embedded within 38 CFR 3.317(d) within the time period specified for the disease in the same table, if a time period is specified or, otherwise, at any time, VA will request a medical opinion as to whether it is at least as likely as not that the condition was caused by the veteran having had the associated disease in column “A” in that same table. See 38 CFR 3.317(d) - Long-term health effects potentially associated with infectious diseases.

Notes

  • When evaluating any claim involving loss or loss of use of one or more creative organs, refer to 38 CFR 3.350 of this chapter to determine whether the Veteran may be entitled to special monthly compensation. Footnotes in the schedule indicate conditions which potentially establish entitlement to special monthly compensation; however, there are other conditions in this section which under certain circumstances also establish entitlement to special monthly compensation.

  • This diagnostic code pertains to renal involvement secondary to all glomerulonephritis conditions, all vasculitis conditions and their derivatives, and other renal conditions caused by systemic diseases, such as Lupus erythematosus, systemic lupus erythematosus nephritis, Henoch-Schonlein syndrome, scleroderma, hemolytic uremic syndrome, polyarthritis, Wegener's granulomatosis, Goodpasture's syndrome, and sickle cell disease.

  • GFR, estimated GFR (eGFR), and creatinine-based approximations of GFR will be accepted for evaluation purposes when determined to be appropriate and calculated by a medical professional.