Mild and moderate Mannose Binding Lectin deficiency are associated with systemic lupus erythematosus and lupus nephritis in Brazilian patients

Perazzio, Sandro Félix; Silva, Neusa Pereira da; Carneiro-Sampaio, Magda; Andrade, Luis Eduardo Coelho

doi:10.1016/j.rbre.2016.01.002

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Revista Brasileira de Reumatologia

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Sumário

Original Articles • Rev. Bras. Reumatol. 56 (3) • May-Jun 2016 • https://doi.org/10.1016/j.rbre.2016.01.002 copy

Mild and moderate Mannose Binding Lectin deficiency are associated with systemic lupus erythematosus and lupus nephritis in Brazilian patients^☆ ☆ Manuscript winner of the "Young scientist prize of the year from the Brazilian Society of Rheumatology-Clinical area" for Sandro F. Perazzio in XXXI Brazilian Congress of Rheumatology, Belo Horizonte, MG, Brazil, 2014.

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Objective

The potential association of mannose binding lectin (MBL) deficiency and systemic lupus erythematosus (SLE) has been investigated in several studies, but results have been mixed. One explanation for the conflicting results could be differences in ethnic background of study subjects. In this study we investigated the association of MBL deficiency and SLE in a large cohort of Brazilian SLE patients and controls.

Methods

Serum MBL and Complement levels were determined for 286 Brazilian adult SLE patients and 301 healthy Brazilian adults as controls. MBL deficiency was classified as mild (<1000 and ≥500 µg/L), moderate (<500 and ≥100 µg/L) or severe (<100 µg/L).

Results

SLE patients presented higher frequency of mild and moderate MBL deficiency compared to controls. SLE patients with MBL deficiency presented higher frequency of lupus nephritis compared to those without MBL deficiency. MBL deficiency was not associated with any other clinical manifestation, use of immunosuppressant therapy, disease activity, disease severity serum or Complement levels.

Conclusion

This study shows that an association between MBL deficiency and SLE does exist in the Brazilian population. We also found an association between MBL deficiency and lupus nephritis. These findings support the hypothesis that MBL deficiency contributes to the development of SLE and lupus nephritis.

Keywords
MBL deficiency; Systemic lupus erythematosus; Immunodeficiency; Lupus nephritis

	n (%)		n (%)
Gender (F:M)^a a Healthy controls: 286:15 (p = 0.856).	271:15	Current immunosuppressant^d d The sum of subjects using each drug results in a number greater than the total number of patients because some subjects used two or more medications.	196 (68.5)
Age (years)^b b Healthy controls: 35.1 ± 11.10 (p = 0.070).	39.29 ± 12.23	• Antimalarials^e e Antimalarial was not considered immunosuppressant.	198 (69.2)
Disease duration (years)	10.58 ± 7.91	• Corticosteroids	153 (53.4)
SLICC-DI (median/25%-75%)	1/0-2	≥20 mg (prednisone)	67 (23.4)
SLEDAI (median/25%-75%)	0/0-2	<20 mg (prednisone)	86 (30.1)
Autoimmune rheumatic diseases	20 (6.9)	• Azathioprine	65 (22.7)
• APS	14 (4.9)	• Methotrexate	48 (16.8)
• Sjögren's syndrome	6 (2.1)	• Cyclophosphamide	23 (8)
Other non-rheumatic autoimmune diseases	14 (4.8)	• Mycophenolate	25 (8.7)
• Hashimoto's thyroiditis	14 (4.8)	• Leflunomide	14 (4.9)
Miscellaneous^c c Miscellaneous: non autoimmune diseases, such as arterial hypertension, dyslipidemia, osteoarthritis, etc. Healthy controls: 43 (14.2%; p < 0.001).	134 (43.8)	• Cyclosporine	7 (2.4)
		• Tacrolimus	4 (1.4)
		• Dapsone	3 (1.0)
		• Thalidomide	2 (0.7)
		Previous immunosuppressant	186 (65)

MBL deficiency	CH50		C2		C3		C4
	Pearson	p	Pearson	p	Pearson	p	Pearson	p
Overall	0.041	0.491	0.118	0.067	0.019	0.751	-0.125	0.233
Mild	0.064	0.657	-0.026	0.857	0.154	0.285	0.215	0.363
Moderate	0.113	0.284	0.204	0.051	0.024	0.819	0.022	0.907
Severe	-0.044	0.807	-0.037	0.839	-0.289	0.102	-0.011	0.979

Clinical features		No MBL deficiency	MBL deficiency
		n = 111	Overall	Mild	Moderate	Severe
		n = 111	n = 175	n = 50	n = 92	n = 33
Gender	M:F	3:108	12:163	2:48	7:85	3:30
Cutaneous	%^a a Percentages refer to the frequency of any given clinical manifestation.	94.6	89.7	92.0	88.0	90.9
Oral ulcers	%	18.9	22.9	32.0	21.7	12.11
Arthritis	%	83.8	88.6	88.0	88.0	90.9
Nephritis	%	47.7	60.6^b b p < 0.05.	74.0^c c p < 0.01.	61.1^b b p < 0.05.	66.7^b c p < 0.01.
II	%	1.3	1.8	2.1	1.5	1.6
III	%	8.6	8.5	8.1	9.6	7.9
IV	%	13.2	12.2	13.6	13.2	13.9
V	%	5.7	6.2	6.1	4.8	5.0
No biopsy available	%	18.9	19.4	18.5	18.9	19.8
Hematologic disease	%	68.5	65.1	60.0	66.3	69.7
Serositis	%	21.6	32.0	24.0	33.7	39.4
Neuropsychiatric	%	18.9	22.9	28.0	20.7	21.2
Recurrent infection	%	9.0	8.6	2.0	10.9	12.11
Other SARD	%	8.1	6.9	4.0	7.6	9.1
NRAID	%	1.8	6.9	12.0	3.3	9.1
Miscellaneous	%	48.6	45.7	56.0	40.2	45.5
Age	Mean ± SD	38.7 ± 11.8	39.2 ± 12.9	38.7 ± 12.1	37.9 ± 13.0	43.3 ± 13.0
Disease duration		9.7 ± 7.1	10.8 ± 8.0	10.3 ± 7.4	11.0 ± 8.5	11.0 ± 7.6
Age at SLE onset		28.9 ± 10.4	28.3 ± 11.0	28.4 ± 10.9	26.8 ± 10.7	32.2 ± 11.14
SLEDAI	Median (min-max)	0 (0-11)	0 (0-12)	0 (0-6)	0 (0-8)	1 (0-12)
SLICC-DI		1 (0-4)	1 (0-5)	1 (0-4)	1 (0-5)	1 (0-3)

		No MBL deficiency	MBL deficiency
		n = 111	Overall	Mild	Moderate	Severe
		n = 111	n = 175	n = 50	n = 92	n = 33
Current immunosupressant ^a a No statistically significant association.	%^b b Percentages refer to the frequency of any given clinical manifestation.	64.0	71.4	68.0	71.7	75.8
Previous immunosupressant ^a a No statistically significant association.	%	63.6	66.7	74.0	63.7	63.6

Corticosteroids
Total^a a No statistically significant association.	%	52.3	54.9	66.0	45.7	57.6
Low doses^a a No statistically significant association.	%	32.4	28.6	32.0	26.1	36.4
High doses^a a No statistically significant association.	%	19.80	25.7	34.0	19.6	21.2

Anti-malarials^a a No statistically significant association.	%	73.0	66.9	70.0	59.8	81.8
Azathioprine ^a a No statistically significant association.	%	21.6	23.40	24.0	25.0	18.2
Mycophenolate ^a a No statistically significant association.	%	9.9	8.0	8.0	6.5	12.1
Methotrexate ^a a No statistically significant association.	%	13.5	18.9	16.0	17.4	27.3
Cyclophosphamide ^a a No statistically significant association.	%	7.2	8.6	6.0	10.9	6.1
Leflunomide ^a a No statistically significant association.	%	5.4	4.6	4.0	3.3	9.1
Dapsone ^a a No statistically significant association.	%	1.8	0.6	2.0	0	0
Thalidomide ^a a No statistically significant association.	%	0.9	0.6	0	1.1	0
Tacrolimus ^a a No statistically significant association.	%	0.9	1.7	2.0	2.2	0
Cyclosporine ^a a No statistically significant association.	%	1.8	2.9	6.0	1.1	3.0

MBL deficiency	SLEDAI		SLICC-DI
	Correlation ^a c p < 0.01.	p	Correlation	p
Overall	-0.068	0.256	-0.05	0.945
Mild	0.037	0.800	-0.090	0.631
Moderate	0.107	0.310	0.163	0.160
Severe	0.036	0.840	-0.322	0.154

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[1] *Corresponding author. E-mail:luis.andrade@unifesp.br (L.E.C. Andrade).