Kazufumi Fujii

Steroid Therapy in IgA Nephropathy: A Retrospective Study in Heavy Proteinuric Cases

29 patients with IgA nephropathy whose proteinuria persisted at a level of 2.0 g/day or more and who received prednisolone treatment for 1-3 years were retrospectively evaluated on their clinical courses. 13 of 14 patients with renal dysfunction of less than 70 ml/min in initial creatinine clearance (Ccr) values subsequently entered a progressive course during a follow-up period of 47 months, leading to end-stage renal failure in 8 cases. On the other hand, only 1 of the other 15 patients with preserved renal function of 70 ml/min or more ended up with end-stage renal failure during a follow-up period of 74 months, although 7 underwent a progressive course. Three patients in the latter group experienced a prominent reduction in proteinuria to less than 1.0 g/day and maintained renal function. Meanwhile, the steroid group of moderate proteinuric patients with a creatinine clearance greater than 70 ml/min had a benign course, while the nonsteroid group had an unfavorable one. These results suggest that steroid therapy in IgA nephropathy may be able to stabilize a progressive course, especially in the early stage of the disease, although, because they come from an uncontrolled study, a definite conclusion cannot be drawn.

Moderately Proteinuric IgA Nephropathy: Prognostic Prediction of Individual Clinical Courses and Steroid Therapy in Progressive Cases

Eighteen of 60 patients with persistent moderate proteinuria between 1.0 and 2.0 g/day and a long-term follow-up of more than 4 years received steroid therapy for a mean period of 18 months. Fifteen of these 18 patients maintained their initial creatinine clearance (CCr) of 70 ml/min or more. The remaining 42 received antithrombocyte drugs and/or nonsteroidal anti-inflammatory drugs, with 31 of them keeping their initial Ccr values of 70 ml/min or more. All 14 cases with initial Ccr values of less than 70 ml/min in both the steroid and nonsteroid groups followed progressive courses, with 12 ending up in hemodialysis. Of the nonsteroid, preserved group of 31 cases, 12 followed a stable course, 10 a progressive course, and the other 9 went into end-stage renal failure necessitating hemodialysis. There were no differences in initial clinical features among these three subgroups. But histological changes were milder in the stable subgroup than in the progressive and hemodialysis subgroups. Further, the total score of eight histological parameters was 6 or less in all but 1 of the cases of the stable subgroup, but 8 or higher in the other two subgroups. Therefore, in the progressive cases alone with initial Ccr values of 70 ml/min or more and high total scores of 7 or more, the long-term clinical courses were compared between steroid and nonsteroid groups (10 and 20 cases, respectively). In the steroid group proteinuria decreased significantly and renal function was preserved well as compared with that in the nonsteroid group.(ABSTRACT TRUNCATED AT 250 WORDS)

Glomerulonephropathy with Amyloid-Stain-Negative Microfibrillar Glomerular Deposits

A 44-year-old man demonstrated proteinuria, microhematuria and renal dysfunction during the course of hyperthyroidism. Neither cryoglobulinemia nor paraproteinemia including light chains was found. No systemic signs suggestive of amyloidosis appeared. Histological findings showed a diffuse mesangial matrix increase with slight mesangial proliferation and diffuse granular depositions of IgG, IgA, C3, C4, Clq, kappa and lambda light chains. Ultrastructurally, microfibrils of about 20 nm in width were seen to be deposited diffusely in mesangial areas and in glomerular basement membranes. Congo red and thioflavin T staining were negative. These findings support the possible existence of a specific glomerular disease different from amyloidosis.

Quantitative analysis of interstitial alterations in lupus nephritis

Ninety-eight patients with lupus nephritis followed up for 3 years or more since initial biopsy were the subject of a qualitative and quantitative study of interstitial lesions. Thirty of the 98 patients showed interstitial cell infiltration and/ or fibrosis. The initial and final creatinine clearance (Ccr) values were significantly lower in these 30 patients than in the remaining cases. Hypertension and progressive cases were more frequent in the group with interstitial lesions. Histologically, the severity and activity of glomerular lesions and IgG deposition in tubular basement membranes were more severe in the interstitial disease group. On a quantitative analysis of interstitial volumes (IV) in 20 of the 30 patients, there were significant differences in Δ IV between the two respective groups with Ccr values greater than 80 ml/min and with those less than 80 both at the initial and final observations (p<0.05, each). Further, renal function was significantly lower in 11 cases showing Δ IV greater than 17%, than in 9 cases with less than this (p<0.01). However, no differences in Δ IV were found between the two groups divided according to the degree of severity and activity of glomerular lesions. Further, there was no correlation between renal function, and the severity and activity of glomerular lesions in the 20 cases with interstitial lesions. These results indicate that quantitative analysis is a very useful tool in the evaluation of the functional and prognostic significance of interstitial alterations in lupus nephritis.

COEXISTENCE OF IgA NEPHROPATHY AND MEMBRANOUS NEPHROPATHY

Renal biopsy findings of a 27‐year‐old female with asymptomatic proteinuria and hematuria revealed two distinct glomerular alterations compatible with both IgA nephropathy and membranous nephropathy. The concomitant presence of two different primary glomerular diseases is very rare.

HYPERSENSITIVITY ANGIITIS WITH GRANULOMATOUS GLOMERULITIS IN A PATIENT WITH PREEXISTING IgA NEPHROPATHY

Following a 6‐year history of asymptomatic proteinuria and microhematuria, a 51‐year‐old man suffered from acute systemic eruption, liver dysfunction and acute renal failure immediately after developing a cold and taking drugs including piroxicam, aspirin and bristocycline. Renal biopsy revealed progressive IgA nephropathy associated with acute tubulointerstitial nephritis and granulomatous glomerulitis. Although the drug actually responsible for this condition was not defined, it is likely that drug‐induced hypersensitivity angiitis with granulomatous glomerulitis was superimposed on preexisting IgA nephropathy in this patient. ACTA PATHOL JPN 38: 209–216, 1988.