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Iga Nephropathy in Systemic Lupus Erythematosus Patients

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Iga Nephropathy in Systemic Lupus Erythematosus Patients r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):270–273 REVISTA BRASILEIRA DE REUMATOLOGIA w ww.reumatologia.com.br Case report IgA nephropathy in systemic lupus erythematosus patients: case report and literature review Leonardo Sales da Silva, Bruna Laiza Fontes Almeida, Ana Karla Guedes de Melo, Danielle Christine Soares Egypto de Brito, Alessandra Sousa Braz, ∗ Eutília Andrade Medeiros Freire School of Medicine, Universidade Federal da Paraíba, João Pessoa, PB, Brazil a r t i c l e i n f o a b s t r a c t Article history: Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has Received 1 August 2014 nephritis as one of the most striking manifestations. Although it can coexist with other Accepted 19 October 2014 autoimmune diseases, and determine the predisposition to various infectious complica- Available online 16 February 2015 tions, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent Keywords: primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear Systemic lupus erythematosus IgA nephropathy fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesan- Glomerulonephritis gial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, show- ing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed con- dition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non-SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in both the short and long terms. © 2014 Elsevier Editora Ltda. All rights reserved. Nefropatia por IgA em paciente portadora de lúpus eritematoso sistêmico: relato de caso e revisão de literatura r e s u m o Palavras chave: O lúpus eritematoso sistêmico (LES) é uma doenc¸a autoimune multissistêmica que tem Lúpus eritematoso sistêmico como uma das manifestac¸ões mais marcantes a nefrite. Apesar de poder coexistir com out- Nefropatia por IgA ras doenc¸as autoimunes e determinar a predisposic¸ão a diversas complicac¸ões infecciosas, Glomerulonefrite o LES raramente é descrito em associac¸ão a nefropatias de etiologia não lúpica. Relatamos ∗ Corresponding author. E-mail: [email protected] (E.A.M. Freire). http://dx.doi.org/10.1016/j.rbre.2014.10.011 2255-5021/© 2014 Elsevier Editora Ltda. All rights reserved. r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):270–273 271 o caso da rara associac¸ão entre LES e nefropatia por IgA (NIgA) primária, a glomerulopa- tia primária mais frequente na populac¸ão mundial. A paciente foi diagnosticada com LES pela ocorrência de eritema malar, alopecia, derrame pleural, proteinúria, pancitopenia, FAN 1:1.280 padrão nuclear pontilhado fino e anticardiolipina IgM 280 U/mL. A biópsia renal rev- elou hipercelularidade mesangial com depósitos isolados de IgA, compatível com NIgA primária. Foi tratada com antimalárico, prednisona e inibidor da enzima conversora de angiotensina e apresentou boa evoluc¸ão. Por consistirem em doenc¸as relativamente fre- quentes, a coexistência de LES e NIgA pode ser de fato um achado incomum por motivos desconhecidos ou uma condic¸ão subdiagnosticada. Este relato atenta para a importância da distinc¸ão entre a atividade de doenc¸a renal do LES e nefropatias não lúpicas, em espe- cial a NIgA, definic¸ão que tem implicac¸ões importantes sobre o prognóstico renal e regimes terapêuticos a serem adotados em curto e longo prazo. © 2014 Elsevier Editora Ltda. Todos os direitos reservados. Introduction Systemic lupus erythematosus (SLE) is a chronic disease of the connective tissue characterized by a number of immuno- logical disorders which result in the onset of inflammatory lesion in various organ tissues. Lupus nephritis (LN) is the most common visceral manifestation of SLE, being diagnosed in approximately 37–45% of the patients at some time dur- 1,2 ing the course of the disease in the Brazilian population. The description of nephritis of other etiologies in patients 3 diagnosed with SLE, however, is an uncommon finding. IgA nephropathy (IgAN), although being the most common cause 4 of glomerulopathy in general population, is rarely associated 5–10 with SLE. We reported a case of rare coexistence of IgAN in a patient with SLE. Case report Forty-year-old female patient, complaining of generalized edema six months ago, associated with polyarthralgias, Fig. 1 – Optical microscopy of specimen obtained by renal intermittent fever, alopecia, weight loss of 7 kg, and ulcerated biopsy revealing mesnagial expansion and hypercelullarity lesion on the right leg. On examination the patient was pale, (HE, 200×). with bilateral periorbital edema, facial flushing, reduced vesicular murmur on the right, lower limb edema (2+/4+) and pyoderma gangrenosum in the middle third of the right leg, associated with stiffness and swelling of the right calf. supplementation of calcium and vitamin D, and antibiotics Additional assessment indicated hemoglobin of 8.9 g/dL, for skin lesion. After initiating treatment, the patient showed leukopenia, mild thrombocytopenia, ANA 1:1.280 of nuclear improvement of the joint, cutaneous, hematologic, and renal fine speckled pattern, CH50 of 88 U/mL, C3 of 46 mg/dL, status, being ready for hospital discharge. C4 of 9 mg/dL, negative anti-dsDNA, anticardiolipin IgM of 211 U/mL, proteinuria of 1045 mg/24 h, endogenous creatinine clearance of 162 mL/min, dysmorphic hematuria, and granular Discussion urinary casts. Chest computed tomography showed bilateral pleural effusion, mild pericardial effusion, and ascites. Lower SLE is a disease marked by heterogeneity of clinical pheno- 1,2 limb Doppler ultrasonography ruled out thrombotic event. types and unpredictable course. These properties make the Renal biopsy was performed, showing 16 intact glomeruli characterization of the disease, the acknowledgment of its with mesangial granular deposition of IgA, negative for other complications, and the detection of overlying conditions a immune deposits, and mesangial hypercellularity (Fig. 1). constant challenge in the routine of rheumatology services. The patient was diagnosed with SLE associated with IgAN, The classic clinical presentation of LN is persistent protein- and she was initially treated with prednisone 60 mg/day, uria and/or cellular casts, or active urinary sediment, i.e., five hydroxychloroquine 400 mg/day, enalapril 10 mg/day, and or more red blood cells or leukocytes per high power field. 272 r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):270–273 Laboratory data may indicate SLE and LN activity, specifi- C4 deposits is not an expected finding in LN, being more com- 5,6,12 cally as high titers of anti-dsDNA antibodies and complement patible with the diagnosis of IgAN. 11 consumption. The distinction between IgAN and LN in SLE patients The IgAN, on the other hand, manifests itself as persistent has important prognostic and therapeutic implications. microscopic, or sporadic macroscopic hematuria, with flare The general recommendations for treatment of IgAN triggered by inflammatory stress. Proteinuria and other find- are focused on blood pressure control and proteinuria ings may or may not be present, and serum complement levels reduction with antihypertensive drugs that act on the 12 are typically normal. renin–angiotensin–aldosterone system, with immunosup- Due to the relative frequency of both conditions, the coex- pression being reserved to cases of crescentic glomeru- istence of primary IgAN and SLE can be an occasional finding. lonephritis, and corticosteroid therapy for limited time only Both LN and IgAN are conditions which are characterized by in patients with persistent proteinuria greater than 1 g/24 h 15 disorders of immune function, with the presence of circulating even after 3–6 months of optimized therapy. On the other immune complexes and anti-C1q antibodies, in addition to the hand, in the treatment for LN, besides controlling protein- 12–14 involvement of genetic and environmental risk factors. uria and blood pressure, base therapy with antimalarials for It is still uncertain whether the rarity of the association of all patients is recommended, as well as specific protocols of SLE with non-lupus nephritis occurs due to a protective factor immunosuppression according to the histopathological class 11 presented by patients with SLE, or if we face underdiagnosed of the disease. 3,8,10 conditions. The coexistence of SLE and IgAN has been Finally, this report highlights the possibility of overex- recently described, with only eight cases published in the posure of primary IgAN in patients with SLE, which is an 5–10 world so far, with this being the first case reported in association that may be more common than it is frequently 5 Brazil. Mac-Moune et al. first reported this association in 1995, described in the literature, and has direct implications on the with three patients who, similar to the one here in Brazil, follow-up and treatment of these individuals with the short-, had glomerular lesion of indolent course associated with exu- medium- and long-term complications. berant systemic presentations. Curiously, Basile et al. and 6,10 Kobak et al. described patients who, besides the diagno- Conflicts of interest sis of IgAN and SLE, had Hashimoto’s thyroiditis, a relatively common association with SLE, but that rarely coexists with IgAN. The authors declare no conflicts of interest.
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