An 8-year-old boy born of non-consanguineous marriage and second birth by order presented with swelling over face for 15 days. Swelling was first noticed in periorbital region and aggravated on waking up followed by abdominal distention, pedal, and scrotal edema. There was no history of breathlessness, jaundice, headache, blurring of vision, rash, arthritis, photosensitivity, or oliguria. Weight on admission was 25 kg compared to 20 kg baseline and BP was normal. Urine analysis showed urine albumin 4 + , urine protein-creatinine ratio 6.9 (normal < 0.2), 2–3 RBC/hpf was normal. Bloodwork showed serum albumin 1.6 g/dl (normal 3.4–5.4 g/dl) and serum triglycerides 293 mg/dl (normal < 150 mg/dl). Diagnosis of nephrotic syndrome was established and patient was started on Tab Prednisolone (60 mg/m2/day) along with strict monitoring urine albumin, daily weight monitoring and BP monitoring. Child showed a response with decreased oedema, improved urine output and serial monitoring showed an improving trend if urine albumin with a value of 2 + after which child was discharged.

However, within 20 days of discharge, patient yet again came in with complaints of generalised body swelling. Urine albumin was 3 + , serum albumin 1.8 g/dl and BP was 134/80 mm Hg, that is in the 95th percentile. In lieu of non-response to steroids and hypertension, investigations were done to rule out secondary causes of nephrotic syndrome. CBC showed normocytic normochromic anaemia, thrombocytopenia, ESR-91, BUN 15 mg/dl, serum creatinine 0.8 mg/dl and urine routine microscopy revealed urine albumin 3 + , urine protein-creatinine ratio 6.0 and 25–30 RBC/hpf. C3 and C4 levels were reduced (41/7), and USG abdomen was suggestive of gross ascites. HIV, HBsAg, HCV, malaria antigen, and dengue IgM screening were all negative. Autoimmune antibody panel was sent showing ANA positive, homogenous pattern 160-fold and nucleolar pattern 80-fold and anti-dsDNA positive (titre 229:24), thus confirming the diagnosis of SLE.

Renal biopsy was subsequently planned in order to stage renal lupus which showed membranous glomerulonephritis with secondary focal segmental glomerulosclerosis corresponding to stage V + III (ISPN stage). Modified activity index was 11 (endocapillary hypercellularity 3, neutrophils 1, fibrinoid necrosis 0, hyaline deposits 1, cellulae/fibrocellular crescents 4, interstitial inflammation 2) and modified chronicity index was 3 (total glomerulosclerosis score 1, fibrous crescents 1, tubular atrophy 1, interstitial fibrosis 0) [3]. Anti-phospholipase A2 receptor (PLA2R) antibody was found to be negative suggesting secondary cause of membranous nephropathy.

Figure 1 shows renal biopsy report depicting diffuse thickening of basement membranes and patent capillary lumina. Few glomeruli show proliferation of mesangial cells (4–6 per region). One glomerulus reveals sclerosis of few of the loops present near the Bowman’s capsule. The interstitium is oedematous and reveals occasional lymphocytes. The tubules show severe hydropic changes and rare tubules contain amorphous material. The vessels are unremarkable. Diagnosis is Membranous Glomerulonephritis with secondary focal segmental glomerulosclerosis.

Histopathology of Renal Biopsy

Diagnosis of SLE was made based on EULAR/ACR criteria requiring positive ANA ≥ 1:80 and a score of 10 points or more (Fig. 2) [4].

EULAR/ACR criteria for Diagnosis of SLE

According to this criterion, our patient had a score of 26 (fever 2, thrombocytopenia 4, renal biopsy class III or IV lupus nephritis 10, low C3, C4 4, and anti-dsDNA antibody 6).

Henceforth the patient was started on induction therapy for SLE according to American Journal of kidney diseases (AJKD) guidelines with hydroxychloroquine, kidney protective therapy in form of ACE inhibitor for proteinuria and mycophenolate moefitil and prednisolone as immunosuppressive therapy. Amlodipine, metoprolol and sublingual nifedipine were given additionally to control hypertension. Proper follow-up plan was advised every week for a month initially and then every month till 1 year. In each visit CBC, urine protein/creatinine ratio, serum creatinine, C3, C4 levels, and ophthalmologic evaluation (to check for hydroxychloroquine toxicity) was done. On follow-up 5 months after initiation of therapy urine protein-creatinine ratio was 2.8, serum creatinine was 0.5 mg/dl, serum albumin was 3.2 g/dl, C3/C4 were 52/7 mg/dl. Subsequent visit 10 months after initiation of therapy showed urine protein-creatinine ratio 0.8, serum creatinine was 0.4 mg/dl, serum albumin 4.0 g/dl, C3 level 100 mg/dl, C4 level 30 mg/dl. He did not progress to end-stage renal disease and did not require dialysis and is doing fine presently.