LSUHSC-S Renal Pathology Consultative Services
Interesting Case
Case Study #:2009 0701
By Stephen M. Bonsib, M.D.
Xin Gu, M.D.
Ami Bhalodia, M.D
318-675-4557 phone
318-675-4541 fax
sbonsi@lsuhsc.ed
Clinical History: 38-year-old Caucasian female with a history of nausea and vomiting of seven days duration presents to the emergency room with renal failure.
Patient denies any history of hypertension. Additional past medical history includes petit mal seizures, depression and headaches. Current medications are 1600mg of ibuprofen per day for the past seven days and Celexa (anti-depressant).
Upon admission, her BP is 109/70, BUN: 116, creatinine 4.25, GFR of 4 and albumin: 3.3. Urine was negative for protein and RBCs
Immunofluorescence and electron microscopy studies revealed no significant findings
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Figure 1: Normal glomerulus
Figure 2: Renal tubules
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Figure 3: Renal tubules
Figure 4: Myoglobin stain
Question:
- What is your clinical diagnosis?
- What is your renal biopsy diagnosis?
- What drugs could explain the interesting finding displayed in the photos?
Answer:
1. Serologies include anti-nuclear antibody (ANA) and rheumatoid factor (because SS frequently occurs secondary to rheumatoid arthritis). Typical Sjögren's syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in Sjögren's. The C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) test results are usually elevated.
2. Renal involvement is reported to occur in 18.4%to 67% of patients with primary Sjögren's syndrome. An inability of the distal renal tubule to secrete hydrogen ions leading to complete or incomplete distal renal tubular acidosis is the most common manifestation of renal involvement. Clinically distal renal tubular acidosis is mostly silent, but there is an increased tendency to stone formation, and some patients may develop nephrocalcinosis and even renal failure. The histopathological renal lesion most often reported in primary Sjögren's syndrome is interstitial nephritis. Glomerular disease is rare, and when it occurs it is often associated with mixed cryoglobulinemia.
3. The renal biopsy reveals a normal glomerulus. However, there is a prominent active interstitial inflammation that affected more than 40% of the cortex, with edema and fibrosis. The infiltrate is composed of plasma cells and occasional lymphocytes, but no eosinophils are seen.
4. The hallmark of Sjögren’s syndrome is infiltration of involved tissue by lymphocytes or plasma cells. Aggregates tend to occur in periductal and periacinar locations and may replace the epithelial structures. Remnants of glands surrounded by large numbers of infiltrating mononuclear cells, lymphoepithelial lesions, can be seen. Glands with significant mononuclear infiltration may also display germinal center formation.
References:
Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). 2002;81: 270.
Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004;164:1275.
Renal involvement in primary Sjögren’s syndrome. Eriksson P, Denneberg T, Larsson L, Lindstrom F. Biochemical markers of renal disease in primary Sjögren’s syndrome. Scand J Urol Nephrol 1995; 29:383–92
Current Rheumatology Diagnosis & Treatment . 2nd edition, 2007
Kelley's textbook of rheumatology . 8th edition, 2008