Journal List > J Rheum Dis > v.20(4) > 1064057

Ryu, Jeon, and Jin: A Case of Behcet's Disease Complicated by IgA Nephropathy

Abstract

Behcet's disease (BD) is a rare, multisystemic disorder characterized by vasculitis. Although renal involvement rarely coexists with BD, several types of renal involvements have been reported: amyloidosis, glomerulonephritis and vascular involvement. Herein, we report a rare case of BD complicated with IgA nephropathy (IgAN). A 42-year-old woman visited the hospital due to joint pains and painful subcutaneous nodules. Based on her medical history of recurrent orogenital ulcers, arthritis, enteral ulcers, erythema nodosum-like skin lesions, and a positive pathergy test, we diagnosed her with BD. To evaluate proteinuria, we performed a renal biopsy. The patient was diagnosed with BD complicated with IgAN, and treated with a low dosage of steroid, colchicine, as well as angiotensin II type I receptor blockers. Although renal involvement in BD is rare, it is important to periodically perform renal function assessments in patients with BD involving abnormal urine results.

References

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Figure 1.
Each glomerulus was mildly increased in size and focally increased in mesangial cellularity (PAS, ×100).
jrd-20-261f1.tif
Figure 2.
Light microscopic finding of renal biopsy. A focus of segmental mesangial expansion by mild mesangial hypercellu-larity is seen with Bowman's adhesion (PAS, ×400).
jrd-20-261f2.tif
Figure 3.
Immunofluorescent finding. Strong positive staining of IgA is noted in the mesangium and focal peripheral capillary loops (DIF for IgA, ×400).
jrd-20-261f3.tif
Figure 4.
Ultrastructural finding. Multiple mesangial electron dense deposits are presentedin the mesangial region with suben-dothelial mesangial interposition (original magnification, ×2,500).
jrd-20-261f4.tif
Table 1.
Reported cases of IgA nephropathy associated with Behcet's disease
Silent glomerular disease Treatment Outcome/Remarks
Diffuse proliferative GN (IgA) (13) None Proteinuria and hematuria persisted
Diffuse proliferative GN (IgA) (14) Colchicine 0.5 mg/d orally Improvement
  Dipyridamole 2∼4 mg/kg/d  
  Cyclosporine 5 to 2.5 mg/kg/d orally (for ocular problems)  
Diffuse proliferative GN (IgA) (3) None Serum creatinine increased 4 years later
  Prednisolone 7.5∼60 mg/d orally (after elevation of serum creatinine) Serum creatinine levels stabilized
Diffuse proliferative GN (IgA) (3) Cepharanthin 20 mg/d orally No change
Diffuse proliferative GN (IgA) (3) Methylprednisolone 24 mg/d Improvement
Focal proliferative GN (IgA) (present) Prednisolone 5 mg/d Improvement after 3 years
  Colchicine 1.2 mg/d, Losartan 50 mg/d  
Focal proliferative GN (IgA) (3) Prednisolone 10∼60 mg orally every other day Improvement
 Nephrotic syndrome    
Diffuse proliferative GN (IgA) (3) Prednisolone 10∼30 mg/d Improvement
  Azathioprine 50 mg/d  
Diffuse proliferative GN (IgA) (2) Candesartan 4 mg/d, Improvement
  Atorvastatin 10 mg/d  
 Acute GN/Rapidly progressive GN  
Focal segmental necrotizing GN (IgA) (15) None Disappearance of proteinuria at 2 months, but hematuria persisted
    Patient also had Henoch-Schö nlein purpura
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