Representative SLE patients with marked elevated autoantibodies without end organ damage (A), with renal disease and resistance to progression to cGN (B and C), and rapidly progressive renal disease leading to end stage renal failure (D)
| Patient | Age | Ethnicity | Initial clinical presentation | Renal disease | Clinical laboratory | Clinical course |
| A | 30 | AAF | Age 16 yr gave birth to infant with neonatal lupus with skin involvement. | None. | +ANA, +anti-Ro, La, Sm and RNP Abs, Normal C2, C4 and CH50. Serum IgG 3700 mg/dl, IgA 560 mg/dl, ESR 110 mm/h, normal CRP, normal creatinine. | Asymptomatic. |
| B | 29 | Korean | At age 18 yr, she developed skin rash, arthritis with positive serology. She was treated with steroids and was non-compliant. | 18 mo after initial presentation, she had trace proteinuria, hematuria. She had skin involvement. At 27 mo, she was found to have marked hematuria, casts, and proteinuria (900 mg per/24 h) with low CI. Renal bx showed class III/V proliferative lupus nephritis. | +ANA, +anti-Ro, La and dsDNA Abs. Marked decrease in C2, C4, and CH50, leukopenia, normal creatinine. | Initial treatment Hydro 400 mg/d, Pred 60 mg/d, and Myco 1,750 mg/d. After 3 mo, no proteinuria and normal C2, C4, and CH50. She became asymptomatic with normal CBC. After 9 mo, Pred was stopped and hematuria was not detected. She has been maintained on Hydro 400 mg/day and Myco 500 mg/d for last six yr without lupus flare. |
| C | 47 | Cauc. | At age 42 yr, she had malar rash, arthralgia, fatigue, and fever with positive ANA and anti-dsDNA. She was treated with Hydro and was in remission. | 4 yr after initial presentation and 3 mo after discontinuing hydroxychloroquine, she presented with marked arthralgia, malar rash, oral ulcers, and diffuse body pain. She was found to have 3+ proteinuria, marked hematuria, and casts in her urine. She had anemia and thrombocytopenia. | +ANA, +anti-Ro, dsDNA Abs + RF, thrombocytopenia, anemia. Normal renal function. | She was treated with Hydro 400 mg/d, Pred 40 mg/d, and Myco 2 g/d. After 2 mo of treatment, her proteinuria ceased. 1 mo later, hematuria was not detected. Her complements returned to normal. Her anemia and thrombocytopenia were cured. She was tapered off Pred and maintained on Hydro 400 mg/d and Myco 500 mg/d without a flare for the past 4 yr. |
| D | 56 | AAF | At age 55 yr, she presented with decreased complements, + ANA, +anti-SmD, +anti-dsDNA, decreased renal function, and CNS disease. Initial renal bx showed class IV proliferative lupus nephritis with moderate CI. She was treated with pulse steroids and monthly high dose Cyclo. | 6 mo after presentation and 2 mo of therapy, she was found to have impaired renal function with creatinine 2.4 and severe proteinuria. Repeated renal bx showed marked sclerotic glomeruli with high CI. | +ANA, +anti-Sm, dsDNA Abs, marked decrease C2, C4, and CH50. Creatinine 2.4 mg/dl. | She was not treated and managed symptomatically. 2 yr later, she was begun on dialysis and 3 yr later, she received a renal transplant. |
| Patient | Age | Ethnicity | Initial clinical presentation | Renal disease | Clinical laboratory | Clinical course |
| A | 30 | AAF | Age 16 yr gave birth to infant with neonatal lupus with skin involvement. | None. | +ANA, +anti-Ro, La, Sm and RNP Abs, Normal C2, C4 and CH50. Serum IgG 3700 mg/dl, IgA 560 mg/dl, ESR 110 mm/h, normal CRP, normal creatinine. | Asymptomatic. |
| B | 29 | Korean | At age 18 yr, she developed skin rash, arthritis with positive serology. She was treated with steroids and was non-compliant. | 18 mo after initial presentation, she had trace proteinuria, hematuria. She had skin involvement. At 27 mo, she was found to have marked hematuria, casts, and proteinuria (900 mg per/24 h) with low CI. Renal bx showed class III/V proliferative lupus nephritis. | +ANA, +anti-Ro, La and dsDNA Abs. Marked decrease in C2, C4, and CH50, leukopenia, normal creatinine. | Initial treatment Hydro 400 mg/d, Pred 60 mg/d, and Myco 1,750 mg/d. After 3 mo, no proteinuria and normal C2, C4, and CH50. She became asymptomatic with normal CBC. After 9 mo, Pred was stopped and hematuria was not detected. She has been maintained on Hydro 400 mg/day and Myco 500 mg/d for last six yr without lupus flare. |
| C | 47 | Cauc. | At age 42 yr, she had malar rash, arthralgia, fatigue, and fever with positive ANA and anti-dsDNA. She was treated with Hydro and was in remission. | 4 yr after initial presentation and 3 mo after discontinuing hydroxychloroquine, she presented with marked arthralgia, malar rash, oral ulcers, and diffuse body pain. She was found to have 3+ proteinuria, marked hematuria, and casts in her urine. She had anemia and thrombocytopenia. | +ANA, +anti-Ro, dsDNA Abs + RF, thrombocytopenia, anemia. Normal renal function. | She was treated with Hydro 400 mg/d, Pred 40 mg/d, and Myco 2 g/d. After 2 mo of treatment, her proteinuria ceased. 1 mo later, hematuria was not detected. Her complements returned to normal. Her anemia and thrombocytopenia were cured. She was tapered off Pred and maintained on Hydro 400 mg/d and Myco 500 mg/d without a flare for the past 4 yr. |
| D | 56 | AAF | At age 55 yr, she presented with decreased complements, + ANA, +anti-SmD, +anti-dsDNA, decreased renal function, and CNS disease. Initial renal bx showed class IV proliferative lupus nephritis with moderate CI. She was treated with pulse steroids and monthly high dose Cyclo. | 6 mo after presentation and 2 mo of therapy, she was found to have impaired renal function with creatinine 2.4 and severe proteinuria. Repeated renal bx showed marked sclerotic glomeruli with high CI. | +ANA, +anti-Sm, dsDNA Abs, marked decrease C2, C4, and CH50. Creatinine 2.4 mg/dl. | She was not treated and managed symptomatically. 2 yr later, she was begun on dialysis and 3 yr later, she received a renal transplant. |
Hydro = hydroxychloroquine, Pred = prednisone, Myco = mycophenolate mofetil, Cyclo = cyclophosphamide, CI = chronicity index, and Cauc. = caucasian.