IgG4-related kidney disease: Pathogenesis, diagnosis, and treatment.

IgG4-related disease (IgG4-RD) is a recently recognized multisystem disease characterized by lymphoplasmacytic inflammation and fibrosis in affected tissues that can affect several organs including the kidney, the involvement of which is often manifested by tubulointerstitial nephritis. The pathogenic mechanisms of IgG4-RD are divided into two sections: one focused on potential initiation mechanisms, particularly genetic, and the other on specific pathological pathways. For the specific pathological pathways, cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RD. Renal involvement may manifest as an intrinsic IgG4-related kidney disease (IgG4-RKD) or as a consequence of ureteric obstruction from retroperitoneal fibrosis. Intrinsic kidney disease is most commonly a tubulointerstitial nephritis, but may also present with a variety of glomerular lesions, in particular membranous nephropathy. The first-line treatment of IgG4-RKD is steroids. The long-term side effects of corticosteroids including diabetes, relapses, and resistance to corticosteroid therapy have prompted some experts to use immunosuppressive agents such as rituximab. However, the pathogenesis remains poorly understood. As any delay in treatment may result in irreversible renal failure, early diagnosis and appropriate therapy are very important. Randomized studies are needed to confirm the efficacy of immunosuppressants such as rituximab.

Renal angiomyolipoma in tuberous sclerosis complex: Case series and literature review.

Tuberous sclerosis complex (TSC) is a genetic disease characterized by the growth of numerous noncancerous tumors in many parts of the body mainly the skin, brain, kidneys. The prevalence of the disease is estimated to be 7 - 12 in 100,000. We report the cases of two black African women diagnosed with TSC at age 25 and 54. They both had renal angiomyolipoma, facial angiofibroma and diffuse hypochromic macules. The older patient remained stable for the 11 years following her diagnosis. But, in the second patient, the disease was more severe with a giant angiomyolipoma, complicated by renal intracystic hemorrhage leading to the patient's death 1 month after diagnosis. Renal involvement can be life-threatening in patients with TSC. The risk of fatal bleeding increases with the size of the tumor. The mTOR inhibitors and angioembolization can improve the prognosis of this disease.

Recurrence or reactivation of SARS-CoV-2 infection after immunosuppressive therapy in patients with ANCA-associated vasculitis and COVID-19.

. Acute tubular injury is the lesion most frequently described in this disease. However, four cases of ANCA-associated vasculitis (AAV) with COVID-19 with pauci-immune glomerulonephritis have recently been described. We report the case of an African woman, aged 70, in whom we diagnosed an AAV with pauci-immune glomerulonephritis in the context of COVID-19. She was treated with hydroxychloroquine and azithromycin for COVID-19. Corticosteroids and cyclophosphamide have been used for the treatment of vasculitis. The evolution was marked by the reappearance of COVID-19 one month after the beginning of an immunosuppressive therapy. The patient died a week later from respiratory failure. The occurrence of AAV during COVID-19 may not be due an unfortunate association but triggered by infection with SARS-CoV-2. The use of immunosuppressive therapy should be discussed due to the potential risk of reactivation or recurrence of the viral infection.

From Hypokalemic Crisis to Sjogren's Syndrome: A Case Report and Literature Review.

Renal involvement occurs in approximately 5% of patients with Sjögren's syndrome (SS). We reported the case of a 20-year-old African woman who was received for paralysis of 4 limbs secondary to hypokalemia. The diagnosis of renal tubular acidosis type 1 complicated by hypokalemia was retained. In the etiologic research of renal tubular acidosis type 1, primary SS was retained. The patient received symptomatic treatment based on potassium chloride, sodium bicarbonate, hydration, and a low protein diet. In terms of etiological treatment, she was put on corticosteroid and hydroxychloroquine. The outcome was favorable with correction of acidosis and hypokalemia.

Proliferative Glomerulonephritis: Risk Factor for Hypertension in Lupus.

Studies report a high prevalence of hypertension in lupus, reaching up to 74%. The incidence of hypertension in SLE patients is increased with the severity of the kidney damage. This work was carried out with the objective of determining the prevalence of hypertension in lupus nephritis and to seek the existence of an association between the presence of a proliferative glomerulonephritis and hypertension. Patients and Methods. This was a case-control study, carried out in the nephrology department of the Aristide Le Dantec University Hospital in Dakar. All records of patients with lupus nephritis over a 10-year period, from January 01, 2007, to December 31, 2016, were included. Results. During the study period, out of 64 lupus nephritis records collected, 28 patients had hypertension, for a hospital prevalence of 43.75%. The mean age of the patients was 30.64 years ± 10.44. There were 24 women and 4 men. The mean systolic blood pressure was 156 mmHg (110-220) and the mean diastolic blood pressure was 100 mmHg (80-130). The mean serum creatinine was 29.48 mg/l ± 24.99. The mean proteinuria was 4.50 g/24 h ± 2.87. Hypertriglyceridemia was observed in one patient. Hypercholesterolemia was present in 3 patients. HDL levels were normal in all patients and elevated LDL levels were noted in all 4 patients. None of our patients had diabetes. Class III was found in 11 cases, class IV in 14 cases, pure class V in 2 cases, and class II in 1 case. Hypertension was associated with the presence of proliferative glomerulonephritis (odds ratio, 7.45; 95% CI, 1.9 to 29.1; p=0.002). Conclusion. Hypertension is common in lupus nephritis. The presence of a proliferative glomerulonephritis is a risk factor for the development of arterial hypertension. Screening and adequate management of hypertension are essential for the prevention of the progression of chronic kidney disease in lupus.

Surgical management of secondary hyperparathyroidism in dialysis patients in Senegal.

Secondary hyperparathyroidism (SHPT) is a common complication of end-stage renal disease. Surgical management occurs in severe forms and/or unresponsive medical treatment. The aim of this study was to outline the indications of parathyroidectomy and its evolution after surgical approach. It was a five-year multicenter backward study in Otorhinolaryngology Department of Fann Hospital and four dialysis centers in Dakar. We include all patients with SHPT who underwent surgery. Preoperative clinical and paraclinical parameters, clinical-biological evolution, and histology findings of the resected parathyroid specimen were collected. Out of 58 patients with hyperparathyroidism, 18 patients required parathyroidectomy, corresponding to a prevalence of 31%. Mean age of patients was 46.6 ± 15.29 years and sex ratio 0.61. Mean duration on dialysis was 44.4 ± 30 months. Ten patients (55.56%) had bone pain and nine patients (50%) had joint pain. Mean serum calcium was 97.27 ± 8.66 mg/L. Mean blood phosphorus levels were 40.47 ± 9.99 mg/L. Mean iPTH rate was 1493.22 ± 1014.93 ng/mL, with a maximum of 5000 ng/mL (77N). Mean value of 25-OH Vitamin D was 32.89 ± 16.02 ng/mL. Parathyroidectomy was indicated after failure of medical treatment with persistence of a serum intact parathyroid hormone concentration above 800 µg/mL in all patients. Subtotal parathyroidectomy (7/8) was performed in 11 patients (61.1%). Two patients (11.11%) benefited from a selective parathyroidectomy (3/4). Evolution was favorable for 13 patients, corresponding to a success rate of 72.2%. It was unfavorable in five patients including one patient with hypoparathyroidism and four patients with recurrent hyperparathyroidism. Surgery for patients with renal hyperparathyroidism in the era of calcimimetics continues to play an important role in selected patients and achieves efficient control of hyperparathyroidism in developing countries.

Evaluation of blood pressure profile in chronic hemodialysis patients using two measurement methods: Home blood pressure measurement and conventional measurement.

The monitoring of hypertension (HTN) in dialysis is often delicate with potentially false measurements due to the white coat effect on the one hand and masked HTN (M-HTN) on the other hand. In this population, there is much controversy over the ideal moment for taking blood pressure (BP) and the target values. An answer to these questions is given by home BP measurement that can detect white coat HTN (WC-HTN) and M-HTN. The aim of this study was to determine the respective prevalence of permanent HTN (P-HTN), WC-HTN, M-HTN, and permanently normotensive (P-NTN) in this population and to analyze the risk factors of M-HTN and WC-HTN in hemodialysis (HD) centers in sub-Saharan Africa. This was a multicenter, descriptive, and analytical cross-sectional study conducted over a period of one month and 23 days. Data collection was performed using a home BP measurement form, conventional BP measurement form, and clinical and laboratory data collection form. The study included all patients who could take their BP at home using an electronic BP machine and record results on the BP forms. All analyses were performed using the Sphinx plus software version 5. The significance level for all statistical tests was set at 5%. The mean age of patients was 45.57 years ± 14.11, with a sex ratio of 1.42. The mean duration in dialysis was 57.96 months ± 34.86. Adherence to the home BP measurement was 100% in 71.7%. P-NTN patients were 15.2% (7 patients), WC-HTN patients were 13% (6 patients), M-HTN patients were 17.5% (8 patients), and P-HTN patients were 54.3% (25 patients). A statistically significant association was observed between WC-HTN and age (P = 0.01). In this work, we noted an important proportion of M-HTN and WC-HTN. This result confirms the need for home BP measurement in the follow-up of BP in HD patients.