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Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

Park C, Choi SW, Kim M, Park J, Lee JS, Chung HC - J Med Case Rep (2014)

Bottom Line: In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema.He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil.A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Ulsan University Hospital, 290-3 Jeonha-dong, Dong-gu, Ulsan 682-714, Republic of Korea. hcjungmd@uuh.ulsan.kr.

ABSTRACT

Introduction: Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported.

Case presentation: A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil.

Conclusions: We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

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Related in: MedlinePlus

Swelling of the proximal interphalangeal joints with swan neck and flexion deformities of both hands.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
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Figure 2: Swelling of the proximal interphalangeal joints with swan neck and flexion deformities of both hands.

Mentions: He was found to be negative for ANA, anti-double-stranded DNA antibody, anti-Smith antibody, anti-SS-A/SS-B, lupus anticoagulant, antistreptolysin O, and antineutrophil cytoplasmic antibody (ANCA). The concentrations of serum IgG, IgA, and IgM were normal. His C3 level was 12.2mg/dL (normal range 90 to 180mg/dL), C4 level <1.5mg/dL (normal range 10 to 40mg/dL), and CH50 level 4.2U/mL (normal range 23 to 46U/mL), and his cryoglobulin test result was negative. His serological test results for hepatitis B and C viruses were also negative. His urine analysis showed trace protein, red blood cell (RBC) count 0 to 1/high-power field (HPF), and a urine protein-creatinine ratio of 364.4mg/g. A chest radiograph showed no abnormalities. An echocardiogram showed moderate mitral and tricuspid regurgitation with moderate-to-severe aortic regurgitation. There was diastolic left ventricular dysfunction and normal left ventricular systolic function. An aortography, superior mesenteric, celiac, and bilateral renal arteriography revealed no abnormalities. High-dose oral prednisolone (1mg/kg) was administered for the treatment of active vasculitis. His abdominal pain and skin lesions subsequently improved.One year after continuous treatment with steroid and azathioprine therapy, he was readmitted due to bouts of repeated deep neck swelling, laryngeal edema, and fever. Symptoms were alleviated with antibiotics and fluid therapy, but no causative organism was identified. After his fever resolved, he developed slow progressive azotemia, proteinuria, and microscopic hematuria. An examination revealed swelling of the proximal interphalangeal joints of both hands with swan neck and flexion deformities (Figure 2). His laboratory tests showed his hemoglobin level to be 8.8g/dL, white blood cell count 4340/μL (neutrophils 71%), platelet count 56×103/μL, total protein level 6.0g/dL, albumin level 3.1g/dL, BUN level 22mg/dL, creatinine level 1.99mg/dL, and C-reactive protein level 2.875mg/L. His C3 level was 9.0mg/dL (normal range 90 to 180mg/dL), C4 level was <1.5mg/dL (normal range 10 to 40mg/dL), CH50 level was <2.0U/mL (normal range 23 to 46U/mL), and C1q level was 5.02mg/dL (normal range 11.8 to 23.8mg/dL). A urine analysis showed protein 1+, red blood cell count 1 to 3/high-power field (HPF), and urine protein-creatinine ratio of 1470.6mg/g. The 24-hour urine protein level was 1.6g/day. A percutaneous renal biopsy was performed; the specimen for light microscopy contained 12 glomeruli and 2 arteries up to interlobular size. The glomeruli were enlarged. The mesangium was focally expanded due to an increase of the matrix without hypercellularity and the glomerular capillary walls were mildly thickened. There were patchy areas of interstitial inflammation with mostly mononuclear cells and fibrosis. The tubules showed degenerative changes of the epithelial cells with proteinaceous casts and atrophy and the blood vessels showed arteriolosclerosis. Immunofluorescent staining revealed granular deposits of IgG(++), IgM(+), C3(+/-), C1q(+/-), and C4d(+++) along the capillary loop. An electron microscopy revealed some irregular electron dense deposits in the subendothelial and paramesangial space. His renal biopsy results were compatible with membranoproliferative glomerulonephritis type I (MPGN type I) (Figure 3A,B). He was diagnosed with HUVS with cardiac valvulopathy, Jaccoud’s arthropathy, and MPGN type I. Three daily doses of intravenous methylprednisolone (500mg each) were administered, followed by oral prednisone (1mg/kg/day) and cyclophosphamide (0.8mg/kg/day).


Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

Park C, Choi SW, Kim M, Park J, Lee JS, Chung HC - J Med Case Rep (2014)

Swelling of the proximal interphalangeal joints with swan neck and flexion deformities of both hands.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4209772&req=5

Figure 2: Swelling of the proximal interphalangeal joints with swan neck and flexion deformities of both hands.
Mentions: He was found to be negative for ANA, anti-double-stranded DNA antibody, anti-Smith antibody, anti-SS-A/SS-B, lupus anticoagulant, antistreptolysin O, and antineutrophil cytoplasmic antibody (ANCA). The concentrations of serum IgG, IgA, and IgM were normal. His C3 level was 12.2mg/dL (normal range 90 to 180mg/dL), C4 level <1.5mg/dL (normal range 10 to 40mg/dL), and CH50 level 4.2U/mL (normal range 23 to 46U/mL), and his cryoglobulin test result was negative. His serological test results for hepatitis B and C viruses were also negative. His urine analysis showed trace protein, red blood cell (RBC) count 0 to 1/high-power field (HPF), and a urine protein-creatinine ratio of 364.4mg/g. A chest radiograph showed no abnormalities. An echocardiogram showed moderate mitral and tricuspid regurgitation with moderate-to-severe aortic regurgitation. There was diastolic left ventricular dysfunction and normal left ventricular systolic function. An aortography, superior mesenteric, celiac, and bilateral renal arteriography revealed no abnormalities. High-dose oral prednisolone (1mg/kg) was administered for the treatment of active vasculitis. His abdominal pain and skin lesions subsequently improved.One year after continuous treatment with steroid and azathioprine therapy, he was readmitted due to bouts of repeated deep neck swelling, laryngeal edema, and fever. Symptoms were alleviated with antibiotics and fluid therapy, but no causative organism was identified. After his fever resolved, he developed slow progressive azotemia, proteinuria, and microscopic hematuria. An examination revealed swelling of the proximal interphalangeal joints of both hands with swan neck and flexion deformities (Figure 2). His laboratory tests showed his hemoglobin level to be 8.8g/dL, white blood cell count 4340/μL (neutrophils 71%), platelet count 56×103/μL, total protein level 6.0g/dL, albumin level 3.1g/dL, BUN level 22mg/dL, creatinine level 1.99mg/dL, and C-reactive protein level 2.875mg/L. His C3 level was 9.0mg/dL (normal range 90 to 180mg/dL), C4 level was <1.5mg/dL (normal range 10 to 40mg/dL), CH50 level was <2.0U/mL (normal range 23 to 46U/mL), and C1q level was 5.02mg/dL (normal range 11.8 to 23.8mg/dL). A urine analysis showed protein 1+, red blood cell count 1 to 3/high-power field (HPF), and urine protein-creatinine ratio of 1470.6mg/g. The 24-hour urine protein level was 1.6g/day. A percutaneous renal biopsy was performed; the specimen for light microscopy contained 12 glomeruli and 2 arteries up to interlobular size. The glomeruli were enlarged. The mesangium was focally expanded due to an increase of the matrix without hypercellularity and the glomerular capillary walls were mildly thickened. There were patchy areas of interstitial inflammation with mostly mononuclear cells and fibrosis. The tubules showed degenerative changes of the epithelial cells with proteinaceous casts and atrophy and the blood vessels showed arteriolosclerosis. Immunofluorescent staining revealed granular deposits of IgG(++), IgM(+), C3(+/-), C1q(+/-), and C4d(+++) along the capillary loop. An electron microscopy revealed some irregular electron dense deposits in the subendothelial and paramesangial space. His renal biopsy results were compatible with membranoproliferative glomerulonephritis type I (MPGN type I) (Figure 3A,B). He was diagnosed with HUVS with cardiac valvulopathy, Jaccoud’s arthropathy, and MPGN type I. Three daily doses of intravenous methylprednisolone (500mg each) were administered, followed by oral prednisone (1mg/kg/day) and cyclophosphamide (0.8mg/kg/day).

Bottom Line: In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema.He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil.A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Ulsan University Hospital, 290-3 Jeonha-dong, Dong-gu, Ulsan 682-714, Republic of Korea. hcjungmd@uuh.ulsan.kr.

ABSTRACT

Introduction: Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported.

Case presentation: A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil.

Conclusions: We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

Show MeSH
Related in: MedlinePlus