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A pathological spectrum of lupus nephritis: A view of 62 cases from a tertiary referral centre
i n d i a n j o u r n a l o f r h e u m a t o l o g y 8 ( 2 0 1 3 ) 5 4 e5 5
Available online at www.indianjrheumatol.com and www.sciencedirect.com
Letter to the Editor
A pathological spectrum of lupus nephritis: A view of 62 cases from a tertiary referral centre Dear Sir, Systemic lupus erythematosis is a multi-systemic disorder where renal involvement is quite common and remains the most dangerous life threatening complication.1 Renal manifestations are highly pleomorphic and patients present with a wide spectrum of symptoms from asymptomatic proteinuria to renal insufficiency warranting dialysis.2,3 We describe histopathological spectrum of 62 biopsy proven cases of lupus nephritis patients at our exclusive nephrourology referral centre. The study consisted of 62 patients, 49 females and 13 males with mean age 29.6 11.6 years at time of presentation. The most common clinical presentation was nephrotic syndrome in 24 patients with median proteinuria of 3.64 g/24 h followed by renal insufficiency in 11 patients while 6 patients presented with rapidly progressive glomerulonephritis, another 8 patients had asymptomatic urinary abnormalities and remaining patients had chronic renal insufficiency. Data shown in Table 1. Pathologically the spectrum of renal lesions in lupus nephritis is broad, involving glomeruli, tubulo-interstitial compartment and blood vessels. Histopathologically ISNRPN class IV was most common subclass in 41 patients followed by 11 patients with class III, 9 patients in class II and 1 patient in class V similar to other studies.4 Class II was marked by presence of pure mesangial hypercellularity of varying degrees with or without increased matrix. There was no segmental or global endocapillary proliferation, necrosis or crescents. The class III lesions were segmental, very rarely
global involving less than 50% of all glomeruli whereas, class IV was characterized by diffuse lesions where greater than 50% of all glomeruli being affected by either segmental (S) or global (G) lesions. These lesions were active (diffuse subendothelial deposits, endocapillary cellular proliferation, inflammatory infiltration, necrosis, crescents) with/without mesangial alterations and/or inactive (sclerosis, scarring). The class V subset of lupus nephritis showed global or segmental subepithelial deposits (greater than 50% of capillaries) with variable mesangial proliferation. The median value of activity and chronicity index were 16.4 (2e20) and 7.6 (2.0e10) in class IV and 12.6 (4e22) and 3.7 (2.0e7) in class III respectively. On immunofluorescence microscopy varying proportions of all immunoglobulins (polyclonal IgG, IgA, IgM) were identified in the immune deposits, IgG was localized almost universally (85e90% of cases), with higher intensity than the others, especially in active lesions. The complement component C3 was seen with a slightly lesser or of equal intensity as IgG in almost all cases along with C1q which was seen mainly in mesangial areas with extension on to capillary walls. This combination of three immunoglobulins and both complement components involved in the immune deposits is termed as “full house” staining pattern seen in immune complex mediated diseases was found in 17 patients (43.5%). Six cases were adolescents aged between 12 and 17 years with active class III and IV lesions and one patient succumbed during dialysis highlighting that lupus nephritis is more common and severe in childhood.5 Lupus nephritis is one of the most important causes of morbidity and mortality in
Table 1 e Showing the demographics and laboratory data.
Number of cases Male/female Age (years) Mean arterial pressure (mm Hg) Serum creatinine (mg/dl) Hemoglobin (g/dl) 24-hour proteinuria (g/day) Hematuria (%) C3 (83e177 mg/dl) C4 (16e47 mg/dl) ANA Anti-ds DNA
Class II
Class III
Class IV
Class V
9 (14.5%) 2/7 28.22 6.6 91.1 1.51 1.1 9.02 3.36 1.59 0.9 07 (77.7)% 110.9 30.5 39.5 3.09 71.4% 57.1%
11 (17.74%) 2/9 29.43 14.64 101.9 1.53 1.22 8.7 1.78 3.32 1.59 9 (81.8)% 107.8 28.1 37 4.65 87.6% 66%
41 (66.1%) 8/31 27.89 8.91 94.1 2.77 1.38 8.36 1.17 4.2 2.8 31(80)% 97.5 17.78 35 7.07 76% 61.9%
1 (0.8%) 1/2 36.33 16.26 96.6 2.90 7.2 0.4 3.25 1.2 01 (33.3)% 79.98 23.53 33.55 12.38 100% 100%
i n d i a n j o u r n a l o f r h e u m a t o l o g y 8 ( 2 0 1 3 ) 5 4 e5 5
patients with SLE. Hence screening for renal disease is imperative in lupus.
Acknowledgments We sincerely acknowledge the kind support of Dr. Shiva Prasad, Prof of Nephrology, and all the staff of Nephrology department at Institute of Nephrourology. We also acknowledge and thank Dr. G K Venkatesh, Director of Institute of Nephrourology for his constant support in all our academic endeavors.
references
1. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15:308e318. 2. Bastian HM, Roseman JM, McGwin Jr G, et al. Systemic lupus erythematosus in three ethnic groups. XII. Risk factors for lupus nephritis after diagnosis. Lupus. 2002;11:152e160. 3. Singh S, Devidayal, Minz R, Nada R, Joshi K. Childhood lupus nephritis: 12 years experience from North India. Rhematol Int. 2006;26:604e607. 4. Chakrabarti S, Ghosh AK, Bose J, De PK, Das K. Clinicopathologic study of lupus nephritis. J Indian Med Assoc. 1998;96:268e271.
55
5. Murali R, Jeyaseelan L, Rajaratnam S, John L, Ganesh A. Systemic lupus erythematosus in Indian patients: prognosis, survival and life expectancy. Natl Med J India. 1997;10:159e164.
Sujatha Siddappa* Laboratory In-Charge, Assistant Professor, Department of Pathology, Institute of Nephrology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India Kowsalya Ramprasad Assistant Professor, Department of Biochemistry, Institute of Nephrourology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India Mythri Kuthagale MuddeGowda Assistant Professor, Department of Microbiology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India *Corresponding author. Tel.: þ91 80 26700527; fax: þ91 80 26706777. E-mail address: [email protected] Available online 15 November 2012 0973-3698/$ e see front matter Copyright ª 2012, Indian Rheumatology Association. All rights reserved. http://dx.doi.org/10.1016/j.injr.2012.11.004
Available online at www.indianjrheumatol.com and www.sciencedirect.com
Letter to the Editor
A pathological spectrum of lupus nephritis: A view of 62 cases from a tertiary referral centre Dear Sir, Systemic lupus erythematosis is a multi-systemic disorder where renal involvement is quite common and remains the most dangerous life threatening complication.1 Renal manifestations are highly pleomorphic and patients present with a wide spectrum of symptoms from asymptomatic proteinuria to renal insufficiency warranting dialysis.2,3 We describe histopathological spectrum of 62 biopsy proven cases of lupus nephritis patients at our exclusive nephrourology referral centre. The study consisted of 62 patients, 49 females and 13 males with mean age 29.6 11.6 years at time of presentation. The most common clinical presentation was nephrotic syndrome in 24 patients with median proteinuria of 3.64 g/24 h followed by renal insufficiency in 11 patients while 6 patients presented with rapidly progressive glomerulonephritis, another 8 patients had asymptomatic urinary abnormalities and remaining patients had chronic renal insufficiency. Data shown in Table 1. Pathologically the spectrum of renal lesions in lupus nephritis is broad, involving glomeruli, tubulo-interstitial compartment and blood vessels. Histopathologically ISNRPN class IV was most common subclass in 41 patients followed by 11 patients with class III, 9 patients in class II and 1 patient in class V similar to other studies.4 Class II was marked by presence of pure mesangial hypercellularity of varying degrees with or without increased matrix. There was no segmental or global endocapillary proliferation, necrosis or crescents. The class III lesions were segmental, very rarely
global involving less than 50% of all glomeruli whereas, class IV was characterized by diffuse lesions where greater than 50% of all glomeruli being affected by either segmental (S) or global (G) lesions. These lesions were active (diffuse subendothelial deposits, endocapillary cellular proliferation, inflammatory infiltration, necrosis, crescents) with/without mesangial alterations and/or inactive (sclerosis, scarring). The class V subset of lupus nephritis showed global or segmental subepithelial deposits (greater than 50% of capillaries) with variable mesangial proliferation. The median value of activity and chronicity index were 16.4 (2e20) and 7.6 (2.0e10) in class IV and 12.6 (4e22) and 3.7 (2.0e7) in class III respectively. On immunofluorescence microscopy varying proportions of all immunoglobulins (polyclonal IgG, IgA, IgM) were identified in the immune deposits, IgG was localized almost universally (85e90% of cases), with higher intensity than the others, especially in active lesions. The complement component C3 was seen with a slightly lesser or of equal intensity as IgG in almost all cases along with C1q which was seen mainly in mesangial areas with extension on to capillary walls. This combination of three immunoglobulins and both complement components involved in the immune deposits is termed as “full house” staining pattern seen in immune complex mediated diseases was found in 17 patients (43.5%). Six cases were adolescents aged between 12 and 17 years with active class III and IV lesions and one patient succumbed during dialysis highlighting that lupus nephritis is more common and severe in childhood.5 Lupus nephritis is one of the most important causes of morbidity and mortality in
Table 1 e Showing the demographics and laboratory data.
Number of cases Male/female Age (years) Mean arterial pressure (mm Hg) Serum creatinine (mg/dl) Hemoglobin (g/dl) 24-hour proteinuria (g/day) Hematuria (%) C3 (83e177 mg/dl) C4 (16e47 mg/dl) ANA Anti-ds DNA
Class II
Class III
Class IV
Class V
9 (14.5%) 2/7 28.22 6.6 91.1 1.51 1.1 9.02 3.36 1.59 0.9 07 (77.7)% 110.9 30.5 39.5 3.09 71.4% 57.1%
11 (17.74%) 2/9 29.43 14.64 101.9 1.53 1.22 8.7 1.78 3.32 1.59 9 (81.8)% 107.8 28.1 37 4.65 87.6% 66%
41 (66.1%) 8/31 27.89 8.91 94.1 2.77 1.38 8.36 1.17 4.2 2.8 31(80)% 97.5 17.78 35 7.07 76% 61.9%
1 (0.8%) 1/2 36.33 16.26 96.6 2.90 7.2 0.4 3.25 1.2 01 (33.3)% 79.98 23.53 33.55 12.38 100% 100%
i n d i a n j o u r n a l o f r h e u m a t o l o g y 8 ( 2 0 1 3 ) 5 4 e5 5
patients with SLE. Hence screening for renal disease is imperative in lupus.
Acknowledgments We sincerely acknowledge the kind support of Dr. Shiva Prasad, Prof of Nephrology, and all the staff of Nephrology department at Institute of Nephrourology. We also acknowledge and thank Dr. G K Venkatesh, Director of Institute of Nephrourology for his constant support in all our academic endeavors.
references
1. Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15:308e318. 2. Bastian HM, Roseman JM, McGwin Jr G, et al. Systemic lupus erythematosus in three ethnic groups. XII. Risk factors for lupus nephritis after diagnosis. Lupus. 2002;11:152e160. 3. Singh S, Devidayal, Minz R, Nada R, Joshi K. Childhood lupus nephritis: 12 years experience from North India. Rhematol Int. 2006;26:604e607. 4. Chakrabarti S, Ghosh AK, Bose J, De PK, Das K. Clinicopathologic study of lupus nephritis. J Indian Med Assoc. 1998;96:268e271.
55
5. Murali R, Jeyaseelan L, Rajaratnam S, John L, Ganesh A. Systemic lupus erythematosus in Indian patients: prognosis, survival and life expectancy. Natl Med J India. 1997;10:159e164.
Sujatha Siddappa* Laboratory In-Charge, Assistant Professor, Department of Pathology, Institute of Nephrology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India Kowsalya Ramprasad Assistant Professor, Department of Biochemistry, Institute of Nephrourology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India Mythri Kuthagale MuddeGowda Assistant Professor, Department of Microbiology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore 560002, Karnataka, India *Corresponding author. Tel.: þ91 80 26700527; fax: þ91 80 26706777. E-mail address: [email protected] Available online 15 November 2012 0973-3698/$ e see front matter Copyright ª 2012, Indian Rheumatology Association. All rights reserved. http://dx.doi.org/10.1016/j.injr.2012.11.004