Minimal Change Nephrotic Syndrome in Two Patients with Suspected Systemic Lupus Erythematosus: Association or Co-Incidence
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Abstract:
Systemic lupus erythematosus (SLE) is characterized by the production of pathogenic autoantibodies and the deposition of immune complexes in various organs, whereas minimal change disease (MCD) is characterized by normal or only mild mesangial cell proliferation without deposition of immune complexes. Although these two diseases are so different, they all share the same characteristics of dysregulation of T-lymphocyte and the appearance of low T4/T8 ratio. In this report, we present two cases who had (suspected) SLE developed minimal change NS (MCNS). Case 1 was diagnosed to have SLE, which fulfilled 5 revised SLE criteria of American College of Rheumatology (ACR), but MCD was shown in renal biopsy. Transient renal insufficiency was also noted. Case 2 had NS as well. Renal pathology revealed MCD and tubuloreticular inclusion. High titer of ANA developed later, and the diagnosis of SLE was highly suspected. Both cases got remission from NS after receiving steroid therapy. Since evidence supports that MCNS might be associated or related with SLE, the therapeutic strategy (corticosteroid) is suggested to treat as primary MCNS. Proper recognition of this entity by renal biopsy should be taken into consideration in SLE patient with NS.Keywords:
Minimal change disease
Immune complex
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Background
Lupus nephritis, a form of glomerulonephritis, is the inflammation of kidneys attributable to systemic lupus erythematosus (SLE). It is an autoimmune disorder in which kidney tissue insult is triggered predominantly by the complement system. Herein, we report the case of a 24 years old female with atypical manifestations of SLE.Methods
Case Presentation: A female aged 24 years was presented with the complaint of fever, cough (with sputum) and dyspnea for the past one month. The preliminary physical examination was suggestive of pneumonia. Her laboratory investigations revealed anisocytosis and normochromic anemia (Hb 9.3 g/dL, HCT 30%). Lymphocytes (12%) were decreased while neutrophils (84%), platelets (439 × 103/µl) and ESR (75 mm/hr) were increased. A serum biochemistry test showed elevated urea (92 mg/dl), creatinine (1.5 mg/dl) and sodium (145 meq/L). The chest X-ray demonstrated a right sided pleural effusion which directed towards a possible Tuberculosis (TB) infection. However, pleural biopsy efficiently excluded an active TB infection. Nephrological investigations exhibited evidence of protein (0.3 g/L) and blood traces in the urine. Her proteinuria (2+) was within nephrotic range which was confirmed through qPCR (3.9 U). Moreover, serum TSH (8.054 mU/L) was also elevated and cardiolipin test was positive for IgM (1.10). SLE specific diagnostic tests anti-dsDNA was positive and ANA was also reactive. Left kidney biopsy exhibited characteristics of diffuse endocapillary proliferative glomerulonephritis. SLE diagnosis was established, and patient was treated with cyclophosphamide pulse therapy along with corticosteroid methylprednisolone and achieved complete remission.Results
CASE REPORTConclusions
To date, this is the first case report of SLE simulating as a TB infection in a developing country. The patient did not display classic triad of SLE; joint pain and malar rash aside from fever. This case reiterates the implication of considering unusual case presentations of SLE and undertaking rigorous clinical workup to minimize the probability of missed cases and improve patient clinical outcomes.Funding Source(s):
NoneAnisocytosis
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Thrombotic microangiopathy
Microangiopathy
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Abstract Background Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases. Methodology A retrospective cohort study conducted on a 10-year registration (2010–2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM). Results Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1–3 relapses during the duration of follow up. Conclusions LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.
Renal pathology
Minimal change disease
Nephrology
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Nephrology
Renal pathology
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A case of systemic lupus erythematosus (SLE) associated with minimal-change nephrotic syndrome (MCNS) is described. A 41-year-old woman with SLE presented with symptoms of nephrotic syndrome. Renal biopsy revealed minor glomerular abnormalities without the deposition of immune complexes. The initial heavy proteinuria promptly decreased after the prednisolone dosage was increased and disappeared 4 weeks later. The patient had a relapse of nephrotic syndrome without exacerbation of immunoserological reactions when the prednisolone dose was subsequently decreased. Remission was achieved 5 days after methylprednisolone pulse therapy. T cell dysfunction, which is present both in SLE and MCNS, might have triggered MCNS during the course of SLE.
Prednisolone
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Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improves patient survival. We present a case of a 49-year-old female with an atypical presentation of LN who initially presented with new-onset hypertension, edema, arthritis, serositis and recently diagnosed leukocytoclastic vasculitis who later developed acute kidney injury, hematuria and nephrotic syndrome. Laboratory testing showed mixed cryoglobulinemia and elevated perinuclear anti-neutrophil cytoplasmic (p-ANCA) and myeloperoxidase (MPO) antibodies. SLE-related serologies were negative. Kidney biopsy showed diffuse proliferative global glomerulonephritis with a full-house nephropathy pattern on immunofluorescence suggestive of LN. Due to high clinical suspicion and renal biopsy findings, she was treated for LN with prompt renal response to immunosuppression. Cryoglobulins, p-ANCA and MPO titers normalized and the negative SLE serologies remained negative. Literature review on antinuclear antibody (ANA)-negative and seronegative LN revealed the following patient presentations: (1) renal-limited or renal and extra-renal manifestations of SLE with negative serologies and (2) renal and extra-renal manifestations of SLE with negative serologies at presentation who develop positive serologies later in follow-up. Both groups represent a unique and challenging cohort of patients who may require longer follow-up and further testing to rule out other glomerular diseases that may mimic LN on renal biopsy. The absence of SLE-related serologies should be weighed against a high pre-test probability of ANA-negative or seronegative LN. If highly suspected, the patient should be treated promptly with close monitoring.
Rapidly progressive glomerulonephritis
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Lupus podocytopathy (LP) is an uncommon manifestation of systemic lupus erythematosus (SLE) and is not included in the classification of lupus nephritis. The diagnosis of LP is confirmed by the presence of diffuse foot process effacement in the absence of capillary wall deposits with or without mesangial immune deposits in a patient with SLE. Here we describe a 13-year-old female who presented with nephrotic syndrome (NS) seven years after the diagnosis of SLE. The renal function had been stable for seven years since the SLE diagnosis, as manifested by the normal serum creatinine, serum albumin and absence of proteinuria. Renal biopsy showed evidence of minimal change disease without immune complex deposits or features of membranous nephropathy. Renal function was normal. The patient had an excellent response to steroid therapy with remission within two weeks. The patient remained in remission five months later during the most recent follow-up. This report highlights the importance of renal histology to determine the accurate etiology of NS in patients with SLE. Circulating factors, including cytokines such as interleukin 13, may play a role in the pathophysiology of LP and needs to be studied further in future larger studies.
Etiology
Pathophysiology
Minimal change disease
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Serositis
Prednisolone
Rapidly progressive glomerulonephritis
Hypoalbuminemia
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Rare cases of association between lupus nephritis (LN) and minimal changes nephrotic syndrome (MCNS) were described. Some authors suggest that this association, taking into account the low prevalence of both diseases, may not be a simple coincidence. Several pathophysiological hypotheses have been proposed to explain this association, including a potential central role of T lymphocytes.We describe the case of a 21 years-old female patient who initially presented with isolated nephrotic range proteinuria. She had no evidence of systemic involvement and Immunological tests were negative, including anti-neutrophil antibodies (ANA) and anti-double-stranded DNA antibodies (Anti-dsDNA). Renal biopsy showed normal glomeruli under light microscopy and no significant deposits were found in immunofluorescence studies. She was diagnosed to have MCNS and responded to a short course of steroids. She remained in remission for three years, after which she presented again with nephrotic-range proteinuria accompanied by clinical signs of systemic involvement. During her second presentation, she fulfilled the diagnostic criteria of systemic lupus erythematosus (SLE) and another kidney biopsy showed class-V lupus nephritis. She was treated with pulse steroids followed by oral prednisolone and mycophenolate mofetil, with good clinical response.This case indicates that relapses of MCNS should be carefully investigated in the right setting to avoid missing a systemic disease such as SLE.
Prednisolone
Nephritis
Minimal change disease
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Two cases with systemic lupus erythematosus (SLE) were treated with high-dose intravenous IgG. In patient 1, the IgG therapy dramatically improved both the lupus nephritis and serological titers for SLE. In patient 2, intravenous IgG yielded a partial remission in the lupus nephritis as well as a satisfactory decrease in proteinuria and disappearance of the skin rash characteristic of SLE. IgG therapy is suggested as possibly beneficial for the treatment of SLE based on different mechanisms from those of corticosteroid action. Further studies are needed to determine the detailed efficacy of IgG therapy for SLE.
Nephritis
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