An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy
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Keywords:
Serositis
Prednisolone
Rapidly progressive glomerulonephritis
Hypoalbuminemia
We report here an adult patient with lupus nephritis who had a good clinical course under long-term observation. Apoptotic bodies in the glomeruli were determined in serial renal biopsy specimens by the nick end labeling method. Apoptotic bodies in the proliferated glomerular cells were detected in the 3rd renal biopsy but not in the 2nd biopsy. The clinical activities of lupus nephritis fluctuated until the time of the 3rd renal biopsy. The 3rd renal biopsy was performed because of increased proteinuria and an increased amount of hyaline, granular and red blood cell casts, with impairment of renal function. The levels of proteinuria, creatinine clearance and serum complements were improved after the 3rd renal biopsy. It appears that apoptosis might control glomerular cell proliferation and also influence the clinical course of lupus nephritis.
Nephritis
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Relapses occur frequently in patients with lupus nephritis. Renal biopsy is the gold standard for assessing renal activity and hence guiding the treatment. Whether repeat renal biopsy is helpful during flares of lupus nephritis remains inconclusive. In the present study, we retrospectively reviewed the patients with lupus nephritis who had more than one renal biopsy with the hope to find the clinical value of repeat biopsy.Patients who had a diagnosis of lupus nephritis and two or more renal biopsies were selected from the database of the patient pathology registration at this renal division. Renal biopsy was evaluated according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis. The pathological patterns and treatment regimens were analyzed after a repeat biopsy.We identified 44 systemic lupus erythematosus patients with serial renal biopsies. In total, there were 94 renal biopsies. Overall, the pathological transition occurred in 64% instances according to the ISN/RPS class. When the transition was analyzed according to proliferative, membranous or mix lesions, it showed different profile: 35% in patients with proliferative lesion, 23.5% patients with mix lesions, 100% in patients with pure membranous lesion. The pathological transition could not be predicted by any clinical characteristics. After the repeat renal biopsy, 34% of patients had a change in their treatment regimens.The pathological conversion was very prevalent in patients with lupus nephritis. However, the transitions became less prevalent when they were analyzed according to pure membranous, proliferative, and mix lesion. Repeat biopsy might be helpful to avoid unnecessary increased immunosuppression therapy.
Renal pathology
Nephrology
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Kidney involvement in lupus occurs in about 60% of patients, and glomerular abnormalities demonstrated in renal biopsy determine most of the signs and symptoms of lupus nephritis, in addition to guiding treatment. We present the uncommon case of male with lupus with refractory renal involvement and difficult to control.
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Lupus nephritis (LN) is a complication of systematic lupus erythematosus (SLE) with significant impact on morbidity and mortality. It is known commonly to affect females but has worse prognosis when males or blacks are affected. In a setting with deficiency in health care delivery, the diagnosis and adequate management of SLE patients becomes difficult and hence less cases of lupus nephritis in black males have been reported. We present a case of a male black patient with SLE who presented with neurolupus, serositis, skin manifestations and Class IV/V lupus nephritis on histology. Our case highlights the challenges in the management of lupus nephritis and the complications with immunosuppression in an attempt to induce remission. There is the need for high index of suspicion for the diagnosis of lupus nephritis especially in black males for prompt management to get the best outcome.
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The natural course of systemic lupus erythematosus (SLE) is characterized by periods of disease activity and remissions. Prolonged disease activity results in cumulative organ damage. Lupus nephritis is one of the most common and devastating manifestations of SLE. In the era of changing therapy to less toxic regimens, some authors have stated that if mycophenolate mofetil can be used for the induction and maintenance treatment in all histological classes of lupus nephritis, renal biopsy can be omitted. This article aims to answer the question of what brings the bigger risk: renal biopsy or its abandonment.
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The gold standard to diagnose the lupus nephritis is the renal biopsy. It provides information not only for diagnosis but also for the treatment plan and the prognosis. Laboratory studies, including the immunological profile, play an essential role in diagnosing and evaluating the lupus nephritis activity. The patient is unlikely to have the active lupus nephritis with the combination of anti-ds DNA, anti-C1q, C3 and C4 being within normal limits. In this case report, we present a patient with moderately active diffuse proliferative lupus glomerulonephritis (Class IV) confirmed by the renal biopsy, while her immunological profile is unusually normal.
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At one time the prognosis of lupus nephritis was reported to be dire, with a 2-year survival of under 10 per cent [1]. This outlook was transformed by the advent of renal replacement therapy and effective immunosuppressive treatment for systemic lupus erythematosus. As a result, concern has focussed on whether patients who are at greatest risk of developing progressive renal failure may be identifiable early in their course, enabling the administration of potentially toxic therapeutic regimens to be limited to those with greatest need. Early histological studies reported an association between the presence of diffuse proliferative nephritis and poor renal outcome [2], in contrast to a relatively good outlook if histological appearances of focal proliferative nephritis or membranous nephritis were present. An association between diffuse proliferative disease and a poor prognosis was confirmed in some [3], although not all subsequent series [4]. Nevertheless, the prognostic value of renal biopsy was questioned by Freis and colleagues [5], who analysed their own and previous experience and claimed that renal histological appearances provided no additional prognostic information over that given by severity of proteinuria and elevation of blood urea. Some degree of renal histological abnormality is present in virtually all patients with SLE, even those without any clinical evidence of nephritis. If the relationship between renal histology and clinical outcome is unpredictable, and if clinical and laboratory variables offer an equally accurate prognosis, should renal biopsy be performed at all in these patients? Studies in the past decade have attempted to answer this question in two ways. Histopathological analyses have tried to define more clearly markers which may be related to prognosis. Those emerging include presence of marked subendothelial deposits on electron microscopy [6], derivation of composite scores of the activity and chronicity of nephritis [7], and presence of tubular atrophy [8], all of which have been associated with poorer outcome. Other studies have examined whether addition of specific histological information may increase the accuracy of predictive models based on clinical variables such as patient age, degree of renal impairment, and proteinuria. In this way, Whiting-O'Keeffe et al. [9] employing step-wise linear regression analysis, found that percentage of sclerotic glomeruli and presence of subendothelia l deposits added to their clinical predictive model, whereas histological classification did not. In 1989 Esdaile and colleagues analysed the long-term outcome in 87 patients with various forms of lupus nephritis [10], using Cox's proportional hazards model. They emphasized the value of poorly-apprec iated clinical markers, such as duration of renal disease, and overall severity of SLE, in determining outcome, and they
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