Focal segmental glomerulonephritis in patients with pulmonary sarcoidosis
Glomerulonefritis focal y segmentaria en paciente con sarcoidosis pulmonar
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We report a relevant case.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">Our patient is a 35-year-old Moroccan male, with no previous illnesses. He sought consultation due to proteinuria. Physical examination without findings. Blood test plasma creatinine 1.1mg/dl, creatinine clearance 97ml/min/1.73m<span class="elsevierStyleSup">2</span>, non-selective glomerular proteinuria 3.67g/d. Albumin 3.9mg/dl, cholesterol 239 mg/dl, urine analysis with proteinuria 300mg/dl and microhaematuria. Red blood cell sedimentation rate 8mmHg 1<span class="elsevierStyleSup">st</span> hour, viral serology, tumour markers, thyroid profile, immunology, protein electrophoresis, serum and urine protein immunoelectrophoresis, hepatopancreatic profile and chest radiograph normal. Renal ultrasound with kidneys of 110 and 114mm and conserved cortex. Renal biopsy (Figure 1) showing 7 glomeruli: 3 sclerotic, 4 with hyaline segmental lesions, cellular proliferation and synechia, mesangial expansion, fibrosis and grade I/III tubular atrophy, lymphoplasmacytic interstitial infiltrates. Arteries and arterioles without lesions. In the direct immunofluorescence: IgM and C'3 deposits in the mesangium. Electron microscopy (EM) was not conducted. When he was diagnosed with FSGN, he was treated with angiotensin converting enzyme inhibitors (ACEI). At 3 months proteinuria had decreased to 1g/d. Dual blockade was started (ACEI and angiotensin II receptor antagonists). At 6 months, the glomerular filtration rate (GFR) remained stable and proteinuria was at 0.5-0.7g/day. At 12 months, he started to experience abdominal pain, diarrhoea, vomiting and increased amylase. Computerised tomography (CT) of the chest and abdomen was performed, revealing lateral cervical, axillary, hilar, mediastinal and liver hilar lymphadenopathy and centrilobular nodules. Bone series and thyroid profile normal, serology for Epstein-Barr virus, cytomegalovirus, rubella, brucellosis, syphilis and toxoplasma and tumour markers negative, angiotensin converting enzyme 46.3U/l (range 20-70), 25OH vitamin D<span class="elsevierStyleInf">3</span> 15.3ng/ml, 1-25 di(OH) vitamin D<span class="elsevierStyleInf">3</span> 45.4ng/ml (range 18-78), intact parathyroid hormone 30pg/ml (range 15-60), urinary calcium 40mg/d, phosphaturia 732mg/d, calcaemia 10mg/dl, phosphataemia 3.0mg/dl. Bronchoscopy with bronchoalveolar lavage and needle aspiration of mediastinal lymph node cellularity showing macrophage predominance. Transbronchial biopsy suggestive of non-necrotising granulomatous inflammatory process, accumulations of histiocytes and giant multinucleated cells. Zhiel-Neelsen and Lowenstein culture negative. Bronchoalveolar lavage with lymphocyte predominant cellularity and increased CD4/CD8 ratio. With sarcoidosis being diagnosed, treatment with prednisone began and was maintained for 12 months. He evolved favourably. CT at 6 months revealed disappearance of mediastinal lymphadenopathy and parenchymal involvement. After 24 months, the GFR remained stable, proteinuria of 0.5g/d was maintained and there were no relevant incidents.</p><p class="elsevierStylePara">The association between sarcoidosis and FSGN dates back to 1978.<span class="elsevierStyleSup">1</span> Only 7 cases are described in PubMed<span class="elsevierStyleSup">1-7 </span>(Table 1). They affect 50% of males, with a mean age of 37.8 years. In all, proteinuria was in the nephrotic range. Renal failure only occurred in 2 cases and both progressed to the end stage, requiring haemodialysis and subsequently a kidney transplant. In all cases, the response to pulmonary symptoms was good, however this was not the case for the evolution of proteinuria, which remained in a nephrotic range in 4, decreased quantitatively in 2 and was resolved in 2 others. GN diagnosis after sarcoidosis diagnosis occurred in 50% of cases. In all biopsies there was glomerular sclerosis, focal segmental lesions and hyalinosis. There was only tubular atrophy in 5, interstitial inflammatory infiltrates in 4, synechiae and increased mesangial matrix in 3, periglomerular fibrosis in 2 and negative direct immunofluorescence in 2 with deposits of IgG in 1, C'3 in 4, IgM in 4 and C'1q in 1 case located in the mesangium and subendothelium. EM was only performed in 2 cases, both revealed podocyte fusion and lack of immune complex. In just one<span class="elsevierStyleSup">2</span> of the cases described, renal interstitial granulomas were found with 12.5% involvement, lower than that reported in literature (15-40%). The good evolution of GFR and proteinuria with conservative treatment was notable and it did not improve by adding corticosteroids for their lung disease.</p><p class="elsevierStylePara">This association can be paradoxical. In sarcoidosis, there is an immune imbalance with upregulation of the TH1 pathway, and in FSGN upregulation of the TH2 pathway.<span class="elsevierStyleSup">8,9</span> However, in late stages TH17 predominates, inducing secretion of interleukin 17<span class="elsevierStyleSup">10</span> favouring the development of fibrosis. Cardiotrophin-like cytokine 1, involved in the development of FSGN, is increased in pathway TH17,<span class="elsevierStyleSup">11-13</span> which may be the link between both.<span class="elsevierStyleSup">11</span></p><p class="elsevierStylePara">We present the case of a patient with FSGN onset who that year was diagnosed with sarcoidosis, with good pulmonary and renal evolution on treatment with corticosteroids and blockade of the renin–angiotensin-aldosterone system.</p><p class="elsevierStylePara"> </p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara"> </p><p class="elsevierStylePara">The authors declare that they have no conflicts of interest related to the contents of this article.</p><p class="elsevierStylePara"><a href="grande/11753_16025_46874_en_11753_t1.jpg" class="elsevierStyleCrossRefs"><img src="11753_16025_46874_en_11753_t1.jpg" alt="Summary of published cases of focal segmental glomerulonephritis associated with sarcoidosis"></img></a></p><p class="elsevierStylePara">Table 1. Summary of published cases of focal segmental glomerulonephritis associated with sarcoidosis</p><p class="elsevierStylePara"><a href="grande/11753_16025_46875_en_11753_f1_copy1.jpg" class="elsevierStyleCrossRefs"><img src="11753_16025_46875_en_11753_f1_copy1.jpg" alt="Focal segmental glomerulosclerosis"></img></a></p><p class="elsevierStylePara">Figure 1. Focal segmental glomerulosclerosis</p>" "pdfFichero" => "P1-E550-S4088-A11753-EN.pdf" "tienePdf" => true "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig1" "etiqueta" => "Tab. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "11753_16025_46874_en_11753_t1.jpg" "Alto" => 1524 "Ancho" => 1449 "Tamanyo" => 349256 ] ] "descripcion" => array:1 [ "en" => "Summary of published cases of focal segmental glomerulonephritis associated with sarcoidosis" ] ] 1 => array:8 [ "identificador" => "fig2" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "copyright" => "Elsevier España" "figura" => array:1 [ 0 => array:4 [ "imagen" => "11753_16025_46875_en_11753_f1_copy1.jpg" "Alto" => 541 "Ancho" => 654 "Tamanyo" => 234375 ] ] "descripcion" => array:1 [ "en" => "Focal segmental glomerulosclerosis" ] ] ] "bibliografia" => array:2 [ "titulo" => "Bibliography" "seccion" => array:1 [ 0 => array:1 [ "bibliografiaReferencia" => array:13 [ 0 => array:3 [ "identificador" => "bib1" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:3 [ "referenciaCompleta" => "Lee SM, Michael AF. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 11 | 8 | 19 |
| 2024 October | 81 | 40 | 121 |
| 2024 September | 99 | 28 | 127 |
| 2024 August | 122 | 73 | 195 |
| 2024 July | 91 | 39 | 130 |
| 2024 June | 102 | 49 | 151 |
| 2024 May | 107 | 32 | 139 |
| 2024 April | 93 | 31 | 124 |
| 2024 March | 103 | 32 | 135 |
| 2024 February | 83 | 43 | 126 |
| 2024 January | 100 | 27 | 127 |
| 2023 December | 62 | 40 | 102 |
| 2023 November | 86 | 31 | 117 |
| 2023 October | 104 | 31 | 135 |
| 2023 September | 120 | 34 | 154 |
| 2023 August | 141 | 18 | 159 |
| 2023 July | 88 | 24 | 112 |
| 2023 June | 87 | 20 | 107 |
| 2023 May | 129 | 35 | 164 |
| 2023 April | 85 | 16 | 101 |
| 2023 March | 109 | 21 | 130 |
| 2023 February | 102 | 18 | 120 |
| 2023 January | 86 | 19 | 105 |
| 2022 December | 106 | 21 | 127 |
| 2022 November | 141 | 27 | 168 |
| 2022 October | 161 | 37 | 198 |
| 2022 September | 109 | 26 | 135 |
| 2022 August | 101 | 29 | 130 |
| 2022 July | 127 | 39 | 166 |
| 2022 June | 113 | 25 | 138 |
| 2022 May | 89 | 23 | 112 |
| 2022 April | 88 | 46 | 134 |
| 2022 March | 130 | 38 | 168 |
| 2022 February | 119 | 30 | 149 |
| 2022 January | 126 | 31 | 157 |
| 2021 December | 110 | 32 | 142 |
| 2021 November | 118 | 40 | 158 |
| 2021 October | 93 | 33 | 126 |
| 2021 September | 132 | 34 | 166 |
| 2021 August | 138 | 33 | 171 |
| 2021 July | 107 | 32 | 139 |
| 2021 June | 117 | 12 | 129 |
| 2021 May | 145 | 31 | 176 |
| 2021 April | 254 | 30 | 284 |
| 2021 March | 179 | 22 | 201 |
| 2021 February | 204 | 30 | 234 |
| 2021 January | 136 | 12 | 148 |
| 2020 December | 95 | 11 | 106 |
| 2020 November | 93 | 14 | 107 |
| 2020 October | 100 | 14 | 114 |
| 2020 September | 95 | 7 | 102 |
| 2020 August | 92 | 13 | 105 |
| 2020 July | 83 | 5 | 88 |
| 2020 June | 54 | 17 | 71 |
| 2020 May | 82 | 14 | 96 |
| 2020 April | 107 | 14 | 121 |
| 2020 March | 91 | 16 | 107 |
| 2020 February | 112 | 13 | 125 |
| 2020 January | 105 | 19 | 124 |
| 2019 December | 122 | 23 | 145 |
| 2019 November | 132 | 25 | 157 |
| 2019 October | 97 | 6 | 103 |
| 2019 September | 72 | 14 | 86 |
| 2019 August | 79 | 10 | 89 |
| 2019 July | 94 | 19 | 113 |
| 2019 June | 69 | 6 | 75 |
| 2019 May | 117 | 12 | 129 |
| 2019 April | 138 | 30 | 168 |
| 2019 March | 103 | 21 | 124 |
| 2019 February | 56 | 15 | 71 |
| 2019 January | 68 | 21 | 89 |
| 2018 December | 145 | 39 | 184 |
| 2018 November | 127 | 15 | 142 |
| 2018 October | 133 | 21 | 154 |
| 2018 September | 127 | 9 | 136 |
| 2018 August | 74 | 20 | 94 |
| 2018 July | 95 | 15 | 110 |
| 2018 June | 68 | 18 | 86 |
| 2018 May | 65 | 18 | 83 |
| 2018 April | 107 | 8 | 115 |
| 2018 March | 69 | 6 | 75 |
| 2018 February | 67 | 4 | 71 |
| 2018 January | 70 | 6 | 76 |
| 2017 December | 67 | 5 | 72 |
| 2017 November | 78 | 21 | 99 |
| 2017 October | 64 | 7 | 71 |
| 2017 September | 58 | 12 | 70 |
| 2017 August | 63 | 12 | 75 |
| 2017 July | 57 | 10 | 67 |
| 2017 June | 58 | 15 | 73 |
| 2017 May | 87 | 6 | 93 |
| 2017 April | 59 | 9 | 68 |
| 2017 March | 39 | 11 | 50 |
| 2017 February | 115 | 16 | 131 |
| 2017 January | 47 | 10 | 57 |
| 2016 December | 55 | 2 | 57 |
| 2016 November | 111 | 5 | 116 |
| 2016 October | 157 | 7 | 164 |
| 2016 September | 270 | 1 | 271 |
| 2016 August | 266 | 4 | 270 |
| 2016 July | 270 | 7 | 277 |
| 2016 June | 186 | 0 | 186 |
| 2016 May | 165 | 0 | 165 |
| 2016 April | 120 | 0 | 120 |
| 2016 March | 93 | 0 | 93 |
| 2016 February | 118 | 0 | 118 |
| 2016 January | 103 | 0 | 103 |
| 2015 December | 140 | 0 | 140 |
| 2015 November | 90 | 0 | 90 |
| 2015 October | 89 | 0 | 89 |
| 2015 September | 76 | 0 | 76 |
| 2015 August | 87 | 0 | 87 |
| 2015 July | 75 | 0 | 75 |
| 2015 June | 44 | 0 | 44 |
| 2015 May | 53 | 0 | 53 |
| 2015 April | 6 | 0 | 6 |
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