Patient with a rash on the legs and acute kidney injury

Rash, AKI, Glomerulonephritis – Nephrology Test

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Background: A male, aged 72, presents to the nephrology clinic with a non-blanching rash on his legs.

The lesions are purple, and they do not fade when pressed with the finger. His blood pressure is elevated, and his creatinine is also elevated above baseline. The u/a reveals protein and blood, consistent with a glomerulonephritis. Serologies are negative, including: MPO-ANCA, PR3-ANCA, ANA, SSA, SSB, and Rheumatoid factor. Peripheral eosinophils are negative. Urine albumin/creatinine ratio is 352.

Collaboration with the patient’s team occurs regarding best practices. The primary provider, nephrologist, dermatologist and rheumatologist, using shared decision making, decide the best next step is to perform a skin biopsy which reveals deposits of IgA and leukocytoclastic vasculitis.

Close up view of skin that will be biopsied to evaluate the patient's vasculitis and glomerulonephritis.
Close up view of skin that will be biopsied to evaluate the patient’s vasculitis and glomerulonephritis.

Please answer the following questions:

Of the following options, which diagnosis is most likely?
* Subacute cutaneous lupus erythematosus
* Microscopic polyangiitis
* Eosinophilic granulomatosis with polyangiitis
* Polyarteritis nodosa
* IgA vasculitis

IgA vasculitis can occur in adults, but it is more common in children.
A
True
B
False

IgA vasculitis is always a mild condition with no severe complications.
A
True
B
False

In men older than 50 years, IgA vasculitis is linked to an increased risk of myelodysplastic syndrome or solid tumors.
A
True
B
False

Eosinophilic granulomatosis with polyangiitis is a small-vessel vasculitis commonly associated with asthma.
A
True
B
False

Microscopic polyangiitis is characterized by immune deposits in the skin.
A
True
B
False

Discussion:

IgA Vasculitis

  • Diagnosis: IgA vasculitis, more severe in adults than in children.
  • Symptoms: Purpuric, non-blanching rash on lower extremities, arthritis, abdominal pain, hematuria.
  • Biopsy Findings: Leukocytoclastic vasculitis with IgA deposits, glomerulonephritis in kidney biopsies.
  • Complications: Aggressive kidney disease, potential transition to diffuse proliferative glomerulonephritis.
  • Association: In men over 50, linked to solid tumors or myelodysplastic syndrome.

Eosinophilic Granulomatosis with Polyangiitis

  • Type: Small-vessel vasculitis.
  • Associated with: Asthma, peripheral eosinophilia, MPO-ANCA

Microscopic Polyangiitis

  • Type: Small-vessel vasculitis affecting arterioles.
  • Features: May involve glomerulonephritis, purpuric skin lesions.
  • Skin: No immune deposits, positive ANCA results.

Polyarteritis Nodosa

  • Type: Medium-vessel vasculitis.
  • Features: Renal artery involvement, hypertension.
  • Skin: No purpura with immune deposits.

Subacute Cutaneous Lupus Erythematosus

  • Presentation: Maculopapular rash on torso and extremities.
  • Tests: Negative ANA, positive Anti-Ro/SSA antibodies.
  • Complications: No glomerulonephritis.

Inspiration: IgA Vasculitis (Henoch-Schönlein Purpura), National Library of Medicine

ACP, MKSAP

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