A 32-year-old male patient with concurrent IgA neph ropathy and ankylosing spondylitis is described. He had HLA-B27 antigen. Renal involvement in seronegative spondylarthropathies is considered to be very rare. Eigh teen such cases reported are reviewed, and a possible common genetic or other pathogenesis discussed.
Case ReportThe patient was a 32-year-old Japanese male who was admitted to hospital because of visual disturbance and mild headache. Posi tive physical findings on admission w'ere a blood pressure of 270/180 mm Hg, a tachycardia of 140 beats/min, grade IV retinopa thy, and scotoma of the left eye. Urinalysis revealed 70 -80 RBC/ HPF, a few hyaline casts and proteinuria of 3.0g/24 h. Laboratory studies revealed an elevated sedimentation rate (ESR) of 31 mm/h, BUN 22 mg/dl, serum creatinine 2.2 mg/dl, a 24-hour creatinine clearance of 36 ml/min and positive HLA-B27. Plasma renin activ ity was 5.7 ng/m l/h. Electrolytes and blood gases were within normal limits. The hematocrit was 36%, hemoglobin 12.2 g/d l with a normal WBC count and differential count. The platelet count was 223,000. Serum IgA concentration was 495 mg/dl which was higher than normal (120-450 m g/dl); the level of circulating immune com plexes was 1.3 pg/ml ( < 5 p/ml). Chest x-ray showed no pulmonary edema and cardiomegaly. The ECG revealed sinus tachycardia and left atrial enlargement. X-ray of sacroiliac joints and spine showed changes typical of ankylosing spondylitis.Malignant hypertension was diagnosed and the patient was rapidly treated, initially with nifedipine 30 mg/day, and then with captopril 100 m g/day; the diastolic blood pressure was controlled at approximately 80 mm Hg. The 24-hour creatinine clearance was between 30 and 40 ml/min. Aortography was performed; there was no evidence of aortitis or stenosis of renal arteries.A renal biopsy demonstrated mild mesangial proliferative glom erulonephritis with focal tubulointerstitial damage. There were three glomeruli of which two showed mild proliferation of mesan gial cell and matrix and one moderate mesangial proliferation. The glomerular basememt membranes were thin and smooth without shrinkage as is frequently seen in hypertensive patients. Focal tubular atrophy with mononuclear infiltrations was observed in the interstitium. Diffuse granular deposits of lgA(2 + ), C3( + ) and fibrinogen ( ± ) were found in the mesangium by immunofluores cence microscopy; (fig. I); deposits of IgG and Clq could not be identified.