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Abstract: PO1488

Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome and IgA Nephropathy

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Cho, Jake Namjik, Maine Medical Center, Portland, Maine, United States
  • Oakley, Grant, Maine Medical Center, Portland, Maine, United States
  • DiPerna, Gary, Maine Medical Center, Portland, Maine, United States
  • Akom, Michael C., Maine Medical Center, Portland, Maine, United States
Introduction

Immunoglobulin A nephropathy (IgAN) is the most common form of glomerulonephritis (GN). It is characterized by IgA-dominant or codominant immune deposits within glomeruli, classically caused by poorly galactosylated immunoglobulin A1 that trigger autoantibodies. Other forms of IgA nephropathy include IgA vasculitis with nephritis (Henoch-Schönlein purpura) and secondary forms arising from chronic liver disease, chronic infections and neoplasms.

Case Description

Patient is a 50 year old female with anemia, hypothyroidism and chronic joint pain who presented for rising creatinine and hematuria. Baseline creatinine was 0.9 mg/dL but increased to 1.8 mg/dL within 2 months. She follows with a rheumatologist for polyarthralgias and hives where labs showed rheumatoid factor of 18 IU/mL, CRP elevated to 125 mg/L, sedimentation rate of 81 mm/hr but normal complement levels and negative ANA titers. Workup for lymphoma or myelodysplasia was negative and she was eventually diagnosed with SAPHO syndrome. Her urinalysis showed active sediment with mixed WBC/RBC casts, dysmorphic RBCs and nephrotic range protein to creatinine ratio of 7.4 g/g Concerning for a rapidly progressive GN, she was admitted to nephrology for expedited biopsy.

Discussion

Labs were negative for pauci-immune GN, phospholipase A2 receptor antibody, hepatitis panel and blood cultures. Kidney biopsy showed a membranoproliferative pattern of injury with IgA dominant GN and diffuse endocapilllary proliferation. This case may represent a rare association between SAPHO and IgA nephropathy which has only been reported in a few case reports. For the proteinuria, she was started on lisinopril and for the IgA, she was given pulse dose steroids and maintenance prednisone. Her creatinine has stabilized to 1.4 mg/dL and urine protein to creatinine ratio has also improved.

Kidney biopsy for IgA case depicting membranoproliferative pattern of glomerular injury in patient with SAPHO syndrome