Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2020  |  Volume : 63  |  Issue : 2  |  Page : 289--291

Hansen's disease with lepra reaction presenting with IgA dominant infection related glomerulonephritis


Elayaperumal Indhumathi, Jeyachandran Dhanapriya, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, Natarajan Gopalakrishnan 
 Department of Nephrology, Institute of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Correspondence Address:
Elayaperumal Indhumathi
Institute of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu
India

Abstract

Various renal abnormalities in leprosy have been described largely in literature but the occurrence of IgA dominant infection related glomerulonephritis in leprosy with type 2 lepra reaction has not been reported so far. We present here a 60-year-old man with a history of leprosy in the past admitted with type 2 lepra reaction, rapidly progressive glomerulonephritis with severe renal failure requiring dialysis and diagnosed to have IgA dominant infection related glomerulonephritis.



How to cite this article:
Indhumathi E, Dhanapriya J, Dineshkumar T, Sakthirajan R, Gopalakrishnan N. Hansen's disease with lepra reaction presenting with IgA dominant infection related glomerulonephritis.Indian J Pathol Microbiol 2020;63:289-291


How to cite this URL:
Indhumathi E, Dhanapriya J, Dineshkumar T, Sakthirajan R, Gopalakrishnan N. Hansen's disease with lepra reaction presenting with IgA dominant infection related glomerulonephritis. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Jul 10 ];63:289-291
Available from: http://www.ijpmonline.org/text.asp?2020/63/2/289/282707


Full Text



 Introduction



IgA dominant infection related glomerulonephritis (IRGN) considered as a variant of acute post infectious glomerulonephritis occurs commonly in elderly diabetic individuals with ongoing infection predominantly staphylococcus aureus. It is characterized by diffuse exudative proliferative glomerulonephritis by light microscopy and intense staining for IgA and C3 deposits by immunoflourescence. It is associated with poor prognosis. Here we report a patient with Hansen's disease and lepra reaction presenting with IgA dominant infection related glomerulonephritis.

 Case Report



A 60-year-old gentleman came to the hospital with history of leg swelling, oliguria, and cola colored urine with worsening of skin lesions all over the body of 10 days duration. He gave history of leg ulceration present for the past 1 week. He did not have history of arthralgia or hemoptysis. There was no history leg swelling in the past. He received treatment for leprosy 2 years back for 6 months. Details of the drugs taken were not available. He was not a diabetic or hypertensive.

On examination he was confused and dyspnoeic. There were multiple hypopigmented anesthetic patches with erythematous plaques seen all over the body and areas of atrophic scar was also present [Figure 1]a. Ulcerations were present over his right leg [Figure 1]b. His ulnar and posterior tibial nerves were thickened. His pulse rate was 110/min and blood pressure was 100/70 mm Hg. Respiratory system examination revealed bilateral basal crackles. Cardiovascular and abdominal examinations did not reveal significant findings. On neurological examination patient was conscious, confused, and moved all the limbs. There was no meningeal signs. Fundus examination was normal. Urine analysis revealed 2+ proteinuria with plenty of red cells. He was pale with hemoglobin of 7.0 g/dL. Total WBC count showed 9800 cells/cumm, ESR of 60 mm in the first hour and peripheral smear showed neutrophils with toxic granulations. His blood urea was 86 mg/dL and serum creatinine was 4.1 mg/dL. Complement levels showed decreased C3 (37 mg/dL) and normal C4 (29.5 mg/dL). ASO titer was 533.0 IU/L. Serum anti-neutrophil cytoplasmic antibodies (ANCA) and anti-GBM antibodies were negative. Urine and blood culture did not show growth. Methicillin-resistant Staphylococcus aureus was grown in wound swab culture. Skin slit smear examination did not reveal acid fast bacilli.{Figure 1}

Skin biopsy showed sheets of foamy histiocytes with numerous lepra bacilli within histiocytes. Occasional nerve bundle is seen with infiltration by histiocytes [Figure 1]c and [Figure 1]d.

Renal biopsy under light microscopy showed enlarged glomeruli with diffuse endocapillary proliferation and influx of neutrophils. Capillary lumens were almost completely obliterated. There was no crescent or necrosis seen [Figure 1]e.

Immunofluorescence staining showed IgA (3+) and C3 (3+) granular positivity over mesangium and capillary walls [Figure 1]f.

A diagnosis of multibacillary leprosy with type 2 lepra reaction and IGA dominant infection related glomerulonephritis (IRGN) was made and he was initiated on Inj. Vancomycin (1 g. IV infusion once in 72 h) and the World Health Organization (WHO) recommended multidrug therapy for multibacillary leprosy with dose modification for renal impairment. (Dapsone 50 mg daily, clofazimine 100 mg daily and 300 mg monthly, and rifampicin 600 mg monthly) along with steroids. He was also started on alternate day hemodialysis. His skin lesions responded well to treatment but he continued to be anuric and dialysis dependent.

 Discussion



Leprosy is an important health problem in developing countries like India. The prevalence rate in India is 0.88/10,000 population.[1] It is a chronic granulomatous disease caused by mycobacterium leprae, an obligatory intracellular organism that preferentially infects peripheral nerves and skin. Renal involvement in leprosy had been reported as early as 1937 by Mitsuda and Ogawa[2] through autopsy findings. They also observed that the renal failure was the cause of death in most of the patients with leprosy. Acute and chronic glomerular, tubulointerstitial lesions had been reported in association with leprosy. Glomerular lesions included mesangioproliferative glomerulonephritis, membranous nephropathy, and membranoproliferative glomerulonephritis. Crescentic glomerulonephritis and amyloid deposits in glomeruli were also reported.[3] Chugh et al. in their analysis of 60 patients with leprosy reported 3 patients with amyloidosis.[4] Mesangial proliferative GN was the common lesion observed in their study. Nakayama et al. in their study demonstrated the epithelioid granuloma with Hansen's bacilli in renal biopsy.[5] Diffuse endocapillary proliferative and exudative glomerulonephritis had been reported in leprosy by Ahsan et al.[6] Electron dense deposits in sub-endothelium were also demonstrated.

The exact mechanism of glomerular involvement is not clear. There is no clear evidence to show the direct involvement of the bacilli though it had been demonstrated in the kidney. Possibly circulating immune complexes that develop during lepra reactions especially erythema nodosum leprosum got deposited in vessels and glomeruli leading to glomerulonephritis.[7]

Acute and chronic tubulointerstitial nephritis, acute tubular necrosis, and tubular dysfunction without histopathological changes had been reported in leprosy.[8] End stage renal disease in leprosy was associated with amyloidosis. Drugs used in the treatment of leprosy such as rifampicin and dapsone had been implicated in interstitial nephritis and intravascular hemolysis. Risk factors for the renal involvement included lepra reaction especially erythema nodosum leprosum in lepromatous leprosy and advanced age.[9]

Our patient was an elderly man with lepromatous leprosy and erythema nodosum leprosum developed diffuse exudative proliferative glomerulonephritis with IgA and C3 immune deposits. IgA dominant IRGN in leprosy has not been reported so far in leprosy. Ahsan et al. in his case report in 1995 reported infection related glomerulonephritis in leprosy with IgG and C3 deposits.[6] IgA dominant IRGN is a new immunopathologic form characterized by diffuse proliferative glomerulonephritis on light microscopy and intense deposits IgA on immunofluorescence.[10] Most cases occur in diabetics and due to staphylococcal infection. Our patient had staphylococcal and mycobacterium leprae infection with IgA dominant IRGN.

To conclude IgA dominant IRGN has to be considered though uncommon in the differential diagnosis in patients with Hansen's disease presenting as rapidly progressive glomerulonephritis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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