Allergy Asthma Respir Dis.  2016 Jan;4(1):70-73. 10.4168/aard.2016.4.1.70.

Eosinophilic granulomatosis with polyangiitis accompanied by rapidly progressive glomerulonephritis

Affiliations
  • 1Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea. allergy21@hotmail.com
  • 2Department of Radiology, Gachon University Gil Medical Center, Incheon, Korea.
  • 3Department of Laboratory Medicine, Gachon University Gil Medical Center, Incheon, Korea.
  • 4Department of Pathology, Gachon University Gil Medical Center, Incheon, Korea.

Abstract

Eosinophilic granulomatosis with polyangitis (EGPA) should be considered in asthmatic patients who present with severe systemic symptoms and eosinophilia. Progressive renal insufficiency can occur during the acute phase of EGPA accompanied by renovascular involvement. A 58-year-old man visited local clinic with complaints of malaise, weight loss, fever, and dyspnea. Eosinophilia was revealed in peripheral blood. Pulmonary function tests were carried out, which yielded decreased lung function with positive bronchodilator response. Kidney and skin biopsies were performed, and histological examination showed acute necrotizing crescentic glomerulonephritis and leukoclastic vasculitis in the skin, which led to a diagnosis of EGPA (Churg-Strauss syndrome) associated with rapidly progressive glomerulonephritis. The patient received pulse steroid therapy with parenteral methylprednisolone, followed by oral prednisolone. Clinical and laboratory findings improved dramatically, and remission was attained rapidly. The patient continued to be in remission for 5 months. Prompt and aggressive treatment with systemic corticosteroids is mandatory to control disease activity and to achieve remission.

Keyword

Churg-Strauss syndrome; Eosinophilic granulomatosis with polyangiitis; Rapidly progressive glomerulonephritis; Kidney injury

MeSH Terms

Adrenal Cortex Hormones
Biopsy
Churg-Strauss Syndrome
Diagnosis
Dyspnea
Eosinophilia
Eosinophils*
Fever
Glomerulonephritis*
Humans
Kidney
Lung
Methylprednisolone
Middle Aged
Prednisolone
Renal Insufficiency
Respiratory Function Tests
Skin
Vasculitis
Weight Loss
Adrenal Cortex Hormones
Methylprednisolone
Prednisolone

Figure

  • Fig. 1 Multiple purpuric papules are observed on both ankles (A), left forearm (B), and buttock (C).

  • Fig. 2 Abdominal computed tomography reveals decreased parenchymal enhancement (arrow) in upper pole of the right kidney.

  • Fig. 3 The glomerulus of kidney biopsy reveals crescentic glomerulonephritis (A), and skin biopsy is consistent with leukocytoclastic vasculitis (B, C). (A) Cellular crescent in glomerulusof kidney (PAS, ×400), (B) neutrophilic infiltration in the superficial dermis with extravasation of red blood cells (RBCs) and intravesicular filling with neutrophils, eosinophils and RBCs (H&E, ×200), and (C) dermal perivascular infiltration of neutrophils, lymphocytes and eosinophils with fibrin material (H&E, ×400).


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