Introduction:
IgA nephropathy commonly presents as asymptomatic hematuria with varying degrees of proteinuria. Electrolyte disturbances as an initial manifestation of IgA nephropathy are exceedingly rare. To date, there are only a few documented cases of Gitelman syndrome coexisting with immune-complex-mediated nephropathy. This case report presents a rare occurrence of the coexistence of Gitelman syndrome and IgA nephropathy, underscoring the potential for unusual clinical presentations in this patient population.
Methods:
A comprehensive review of the patient's medical records, including clinical presentation, laboratory findings, treatment, and follow-up data, was conducted.
Results:
A 20-year-old male presented to our outpatient department with complaints of blurred vision and headache. His initial blood pressure was 200/100 mm Hg. Apart from a 5-year history of smoking, there was no significant past medical history. Physical examination was unremarkable, with blood pressure readings consistently elevated in all four limbs. Fundoscopic examination revealed Grade III hypertensive retinopathy with papilledema, and echocardiography showed concentric left ventricular hypertrophy.
Hematological parameters were within normal limits. Biochemical evaluation revealed hypokalemia (serum potassium: 2.5 mmol/L) and metabolic alkalosis (pH: 7.50, pCO2: 47 mmHg, HCO3-: 30 mmol/L, BE: +7). Plasma aldosterone was elevated (18.30 ng/dl), with markedly increased direct renin levels (>500 µIU/ml). Ultrasound renal Doppler findings were normal. Liddle syndrome was excluded due to the presence of hyperreninemia. A contrast-enhanced CT of the abdomen was unremarkable. Further investigations identified hypocalcemia (7.2 mg/dl) with normal serum magnesium (1.7 mg/dl) and phosphorus (3.2 mg/dl) levels. Both 24-hour urinary calcium (86 mg) and magnesium (43 mg/dl) excretion were reduced, consistent with a Gitelman phenotype. Urinalysis revealed nephrotic-range proteinuria with active sediment, while other laboratory values were within normal limits.
A renal biopsy demonstrated mesangial (M1) and endocapillary proliferation (E1), with no evidence of segmental sclerosis (S0) or crescents (C0). The degree of interstitial fibrosis and tubular atrophy was less than 10% (T0). Immunofluorescence showed dominant mesangial IgA deposits.
The patient was managed with telmisartan and eplerenone, along with potassium and calcium supplementation. Corticosteroids were initiated due to persistent proteinuria and tapered off over six months. At the latest follow-up, the patient achieved complete remission with normalization of electrolyte levels.
Conclusions:
Although rare, the coexistence of IgA nephropathy and Gitelman syndrome has been documented. It is imperative to consider renal biopsy in cases of Gitelman syndrome presenting with significant proteinuria, particularly nephrotic-range proteinuria, to elucidate the underlying pathology. Further studies are required to determine whether this coexistence is merely coincidental or suggests a potential pathophysiological association.
I have no potential conflict of interest to disclose.
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