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

• Kim S
• Lee SM
• Shim YS
• Ahn JY
• Lee S
• Ha SH
• Lee SP

• KMID: 2218623
• DOI: http://doi.org/10.4168/aard.2016.4.1.70

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).

Reference

1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951; 27:277–301.

2. Oh MJ, Lee JY, Kwon NH, Choi DC. Churg-Strauss syndrome: the clinical features and long-term follow-up of 17 patients. J Korean Med Sci. 2006; 21:265–271.

3. Baldini C, Della Rossa A, Grossi S, Catarsi E, Talarico R, d'Ascanio A, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 38 patients from a single Italian centre. Reumatismo. 2009; 61:118–124.

4. Jeong IH, Nam YH, Kim DC, Lee HW, Yoon NB, Um SJ, et al. A case of Churg-Strauss syndrome with acute renal failure. Korean J Asthma Allergy Clin Immunol. 2012; 32:193–197.

5. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990; 33:1094–1100.

6. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999; 78:26–37.

7. Solans R, Bosch JA, Perez-Bocanegra C, Selva A, Huguet P, Alijotas J, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001; 40:763–771.

8. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984; 63:65–81.

9. Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant. 1990; 5:161–167.

10. Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis. 2006; 47:770–779.

11. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. 1977; 52:477–484.

12. Rychlík I, Tesar V, Stejskalova A, Stejskal J, Honsova E, Bartůnková J. ANCA-positive Churg-Strauss syndrome with renal failure. Nephrol Dial Transplant. 1997; 12:837–838.

13. Gaskin G, Clutterbuck EJ, Pusey CD. Renal disease in the Churg-Strauss syndrome: diagnosis, management and outcome. Contrib Nephrol. 1991; 94:58–65.

14. Prochnicka A, Zelichowski G, Wojtecka A, Lubas A, Bant A, Perkowska-Ptasinska A, et al. Atypical clinical presentation of Churg-Strauss syndrome with rapidly progressive glomerulonephritis: case report. Pol Arch Med Wewn. 2009; 119:830–833.

15. Sugino K, Kikuchi N, Muramatsu Y, Sano G, Kusano E, Isobe K, et al. Churg-Strauss syndrome presenting with diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Intern Med. 2009; 48:1807–1811.

16. Yamashita Y, Yorioka N, Taniguchi Y, Yamakido M, Watanabe C, Kitamura T, et al. Nonasthmatic case of Churg-Strauss syndrome with rapidly progressive glomerulonephritis. Intern Med. 1998; 37:561–563.

17. Tesar V, Rychlik I, Bartunkova J, Stejskalova A, Honsova E, Lachmanova J. The Churg-Strauss syndrome with rapidly progressive glomerulonephritis positive for antineutrophil cytoplasmic antibodies. Cas Lek Cesk. 1993; 132:691–694.