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Year : 2014  |  Volume : 1  |  Issue : 2  |  Page : 108-109

Immunoglobulin a nephropathy presenting as malignant hypertension: A less common scenario

Department of General Medicine, IPGMER, Kolkata - 700 020, West Bengal, India

Date of Web Publication16-Jul-2014

Correspondence Address:
Subrata Chakrabarti
Doctor Hostel, IPGMER, AJC Bose Road, Kolkata - 700 020, West Bengal
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DOI: 10.4103/2384-5147.136825

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How to cite this article:
Chakrabarti S. Immunoglobulin a nephropathy presenting as malignant hypertension: A less common scenario. Sub-Saharan Afr J Med 2014;1:108-9

How to cite this URL:
Chakrabarti S. Immunoglobulin a nephropathy presenting as malignant hypertension: A less common scenario. Sub-Saharan Afr J Med [serial online] 2014 [cited 2021 Jan 19];1:108-9. Available from: https://www.ssajm.org/text.asp?2014/1/2/108/136825


Malignant hypertension is a potentially life-threatening condition characterised by acute severe rise in blood pressure (BP) associated with papilledema and other manifestations such as hypertensive encephalopathy, acute renal failure, and pulmonary edema. Immunoglobulin A (IgA) nephropathy can lead to malignant hypertension although rarely.

A 47-year-old female patient presented with sudden onset bifrontal headache, vomiting, and blurred vision for 1 day prior to admission. There was no history of previous hypertension or fever or altered sensorium or seizure or head trauma or diplopia or photophobia or neck pain or hematuria. On examination, the patient was anxious, had mild pallor, tachycardia (heart rate 112 bpm), increased respiratory rate (28/min). She was found to have a BP of 214/116 mm of Hg with no postural variation. Ophthalmoscopy revealed bilateral severe papilledema with macular star formation and narrowed tortuous vessels and flame shaped hemorrhages and soft exudates in both eyes in both eyes (stage 4 hypertensive retinopathy according to Keith-Wagner-Barker classification) [Figure 1]. The hypertensive emergency was managed with intravenous labetalol. Urgent computed tomography brain was done which showed no ischemic or hemorrhagic event. Routine investigations revealed anemia (hemoglobin: 9.2 g/dl), dysmorphic red cells on peripheral blood smear, serum creatinine: 3.7 mg/dl, normal serum electrolytes. Urinalysis showed significant albuminuria (+++) with microscopic hematuria. His 24 h urinary protein excretion was 4.2 g. Serum albumin level was 3.6 g/dl. Urinary metanephrine level was within normal limits. Antinuclear antibody, C3, C4, cytoplasmic-antineutrophil cytoplasmic antibody showed no abnormal results. Ultrasonography of abdomen showed bilateral renal parenchymal disease (right kidney: 82 mm, left kidney: 87 mm). Renal artery Doppler study was normal (no evidence of renal artery stenosis). The patient required treatment with frusemide, ramipril, amlodipine and prazosin for control of BP. Renal biopsy [Figure 2] showed IgA nephropathy (mesangial hypercellularity: M1, endocapillary proliferation: E1, segmental sclerosis: S1, tubular atrophy/interstitial fibrosis: T0, no global glomerulosclerosis). There was + 3 mesangial positivity for IgA and + 1 mesangial positivity for C3 [Figure 3]. Stainings for IgG, IgM and C1q were negative.
Figure 1: Ophthalmoscopy revealing severe papilledema with fl ame shaped haemorrhages and soft exudates and narrowed tortuous blood vessels suggestive of advanced hypertensive retinopathy

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Figure 2: Light microscopic description of renal biopy samples stained with special stain (periodic acid-Schiff, silver and trichrome). Fourteen glomeruli are included in this biopsy of which two are globally sclerotic. Segmental sclerosis with adhesion to the Bowmans capsule are identifi ed in three glomeruli. Fibrocellular crescent and segmental endocapillary formation is present in three glomeruli. All the glomeruli show an increase in cellularity and mesangial matrix. Interstitial fi brosis, tubular atrophy and tubular loss involves <20% of the core. There is mild arteriolar hyalinosis and fi brointimal proliferation of the arteries

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Figure 3: Imunofl uorescence staining of the renal biopsy samples: Three glomeruli are present for evaluation. The sections are stained for immunoglobulin G (IgG), IgM, IgA, C3, C1q, kappa and lambda light chains. There is + 3 mesangial positivity for IgA and + 1 mesangial positivity for C3. Stains for IgG, IgM and C1q are negative

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Immunoglobulin A nephropathy usually manifests as episodic macroscopic hematuria following upper respiratory tract infection (synpharyngitic hematuria) or occasionally asymptomatic proteinuria with microscopic hematuria. Malignant hypertension is however a rare presenting feature. [1] Here, the index patient presented with malignant hypertension. Zhang et al. demonstrated that the occurrence of malignant hypertension in IgA nephropathy was not associated with the background glomerular pathological phenotypes of IgA nephropathy. However, Anti-endothelial cell antibodies might play a role in the pathogenesis. [2]

Our patient also had a notable feature of normoalbuminemia with absence of edema in the presence of nephrotic range of proteinuria. Similar finding was also reported by Goswami et al. [3] Among patients presenting with nephrotic range proteinuria, normal albumin level in serum is usually associated with IgA nephropathy. Although not exactly known, it may be due to lower fraction of albumin in urinary protein and normal capillary permeability in patients with IgA nephropathy. [4]

  References Top

1.Barratt J, Feehally J. IgA nephropathy. J Am Soc Nephrol 2005;16:2088-97.  Back to cited text no. 1
2.Zhang JJ, Xu LX, Liu G, Zhao MH, Wang HY. The level of serum secretory IgA of patients with IgA nephropathy is elevated and associated with pathological phenotypes. Nephrol Dial Transplant 2008;23:207-12.  Back to cited text no. 2
3.Goswami RP, Sinha D, Mondal S, Mandal S, Ete T, Nag A, et al. Malignant hypertension and nephrotic range proteinuria without hematuria: IgA nephropathy. Indian J Nephrol 2013;23:390-2.  Back to cited text no. 3
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4.Chen M, Zhou FD, Zhao MH, Wang HY. Normoalbuminaemia is associated with IgA nephropathy in primary glomerulopathy with nephrotic-range proteinuria in Chinese patients. Nephrol Dial Transplant 2011;26:1247-52.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]


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