Autoimmune polyglandular syndrome type III associated with antineutrophil cytoplasmic autoantibody-mediated crescentic glomerulonephritis: A case report and literature review.

RATIONALE: Polyglandular autoimmune syndromes (PAS) are a heterogeneous group of rare diseases characterized by the association of at least 2 organ-specific autoimmune disorders, concerning both the endocrine and nonendocrine organs. Type III is defined as the combination of autoimmune thyroid disease and other autoimmune conditions (other than Addison disease), and is divided into 4 subtypes. We describe a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, antineutrophil cytoplasmic antibody (ANCA)-mediated crescentic glomerulonephritis, and hyperparathyroidism. Co-occurrence of these 5 diseases allowed us to diagnose PAS type IIIc. The rare combination of these different diseases has not been reported before. PATIENT CONCERNS: A 51-year-old woman was admitted in April, 2019 after the complaint of an enlarged thyroid. She was diagnosed with Hashimoto thyroiditis at the age of 36. At age 40, she was diagnosed with an adult-onset Still disease. Three months before admission, she experienced renal insufficiency. After admission, she was diagnosed with hyperparathyroidism. DIAGNOSIS: Renal biopsy revealed renal vasculitis and crescentic nephritis. Antineutrophil cytoplasmic autoantibody showed that human perinuclear ANCA and myeloperoxidase ANCA were positive. Therefore, the patient was diagnosed with vasculitis and ANCA-mediated crescentic glomerulonephritis. After admission, parathyroid single-photon emission computed tomography/computed tomography fusion image demonstrated the presence of hyperparathyroidism. INTERVENTIONS: The patient was treated with high-dose methylprednisolone pulse therapy (0.1 g/d) for vasculitis and ANCA-mediated crescentic glomerulonephritis, calcium and vitamin D3 (600 mg/d elemental calcium [calcium carbonate] and 2.5 µg/d active vitamin D3) for hyperparathyroidism, and levothyroxine sodium (50 ug/d) for Hashimoto thyroiditis. OUTCOMES: Up to now, serum thyroid-stimulating hormone, total triiodothyronine, total thyroxine, free triiodothyronine, and free thyroxine were within the normal ranges. Patient's renal function did not deteriorate. LESSONS: We report a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, ANCA-mediated crescentic glomerulonephritis, and hyperparathyroidism, which is a very rare combination. We present this case as evidence for the coexistence of several different immune-mediated diseases in the clinical context of a PAS IIIc.

Individualised Homeopathic Therapy in ANCA Negative Rapidly Progressive Necrotising Crescentic Glomerulonephritis with Severe Renal Insufficiency - A Case Report.

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-negative Rapidly Progressive Glomerulonephritis (RPGN) is a severe form of autoimmune renal injury with a bleak prognosis. A 60-year-old Indian woman was treated with classical homeopathy for ANCA-negative RPGN, and after one year of treatment, serum creatinine and other parameters indicating renal injury dropped steadily despite the withdrawal of immunosuppressive drugs; renal dialysis, which was conducted twice a week initially, was made rarer and stopped after one year. Classical homeopathy may be considered a potential therapeutic modality in severe pathologies. Controlled studies are required to establish further the extent to which classical homeopathy may relieve patients from procedures such as dialysis that cause considerable physical and economic discomfort.

Unusual case of chronic recurrent multifocal osteomyelitis.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory bone disorder that primarily affects young girls, with a mean age of 10 years at onset. Generally, it is a self-limited disease. However, recent data indicate that more than 50% of patients have a chronic persistent disease and about 20% a recurring course of this condition. Also, there are more cases reported with associated auto-inflammatory and autoimmune diseases. In this case report, we present a rare case of sporadic CRMO in which the patient eventually developed C-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies)-associated renal vasculitis and hyperparathyroidism. CASE PRESENTATION: A 14 year old female patient was brought to the emergency department with a sudden onset of left leg pain and oedema. After physical evaluation and initial investigation, she was diagnosed with femoral and pelvic deep vein thrombosis. While searching for possible thrombosis causes, osteomyelitis of the left leg was identified. Additional CT and MRI scans hinted at the CRMO diagnosis. Due to the multifocal lesions of CRMO, endocrinological evaluation of calcium metabolism was done. The results showed signs of hyperparathyroidism with severe hypocalcaemia. Moreover, when kidney damage occurred and progressed, a kidney biopsy was performed, revealing a C-ANCA associated renal vasculitis. Treatment was started with cyclophosphamide and prednisolone according to the renal vasculitis management protocol. Severe metabolic disturbances and hyperparathyroidism were treated with alfacalcidol, calcium and magnesium supplements. Secondary glomerulonephritis (GN) associated hypertension was treated with ACE (angiotenzine converting enzyme) inhibitors. Anticoagulants were prescribed for deep vein thrombosis. After 1.5 years of treatment, the patient is free of complaints. All microelement and parathormone levels are within normal range. Kidney function is now normal. To date, there are no clinical or diagnostic signs of deep vein thrombosis. CONCLUSIONS: This case report presents a complex immunodysregulatory disorder with both auto-inflammatory and autoimmune processes. We hypothesize that the long lasting active inflammation of CRMO may induce an autoimmune response and result in concomitant diseases like C-ANCA-associated vasculitis in our patient. Any potential specific pathogenic relationships between these two rare pathologies may need to be further studied. Furthermore, there is a lack of specific biomarkers for CRMO and more studies are necessary to identify CRMO's characteristic patterns and how to best monitor disease progression.

Association between plasma phosphorus and renal outcome: A prospective cohort of patients majorly with glomerulonephritis.

AIM: Studies investigating the association between blood phosphorus and renal outcomes yielded inconsistent results, and studies from Asian population are extremely limited. We initiated the present cohort study, aiming to prospectively examine the association between blood phosphorus and adverse renal outcomes in a prospective chronic kidney disease (CKD) cohort of Chinese patients majorly with glomerulonephritis. METHODS: A total of 1430 patients were involved in the study. Linear regression analyses were used to assess the relationship between phosphorus and the slope of estimated glomerular filtration rate (eGFR). Cox regression analyses were used to assess the association between phosphorus and composite outcomes, which were defined as the presence of at least one of: eGFR halving, end stage renal disease, or death. RESULTS: During follow-up for an average of 41.4 months, 196 patients developed composite outcomes. The time-average plasma phosphorus was independently associated with the slope of eGFR (ß = -0.18, 95% CI: -4.42 to -2.19, P < 0.001). Each 1 mg/dL increases of baseline and time-average phosphorus were respectively associated with a 1.33 (95% confidence interval (CI): 1.09-1.63; P = 0.005) and 2.79 (95%CI: 2.21-3.52; P < 0.001) fold higher risk of composite outcomes. Compared with participants in the bottom quartile of time-average phosphorus, those in the top quartile were at increased risk of composite outcomes, with a hazard ratio of 6.52 (95% CI: 3.05-13.90; P < 0.001). CONCLUSION: Plasma phosphorus level is an independent risk factor of adverse renal outcomes in Chinese CKD patients majorly with glomerulonephritis. Compared with baseline value, time-average phosphorus has a stronger relationship with renal prognosis.

[Discussion of Syndrome Elements for Chronic Glomerulonephritis on the Basis of Factor Analysis Method].

Objective To preliminarily observe syndrome elements of chronic glomerulonephritis by using factor analysis method. Methods Data of four diagnostics were collected from 289 chronic glo- merulonephritis outpatients and inpatients at Dongzhimen Hospital between February 2011 and February 2012. Under the guidance of Chinese medicine ( CM) , the distribution features of syndrome elements of chronic glomerulonephritis were preliminarily observed using factor analysis method. Results The primary locations of syndrome element were Pi and Shen, and followed by Gan and Fei. The primary nature of syndrome elements were qi deficiency, yin deficiency, and yang deficiency. The primary pathogenic factors of syndrome elements were water dampness, heat, and blood stasis. Conclusions Using factor analysis method, syndrome elements of chronic glomerulonephritis were primarily summarized as four aspects : dampness, heat, stasis, and deficiency. Of them , deficiency was an important pathogenic fac- tor. Sthenic syndrome was seldom seen. Dampness was an important pathogenic factor.

Crescentic acute glomerulonephritis with isolated C3 deposition: a case report and review of literature.

An eight-year-old girl, presenting with palpebral edema, gross hematuria, and foam in urine, was admitted to our hospital. Investigations indicated increased serum antistreptolysin O (ASO) and anti-mycoplasma antibody titers. Renal biopsy showed crescentic poststreptococcal acute glomerulonephritis (CPAGN) with isolated C3 deposition in the glomeruli. Electro-microscope examination showed subepithelial deposition of electron dense material. She received the double pulse therapies of methylprednisolone and cyclophosphamide as well as the treatment of oral prednisolone, angiotensin converting enzyme-II (ACE-II) inhibitor, dipyridamole and traditional Chinese medicine. The complete clinical remission was achieved after 9 months. No serious adverse effects were observed during the follow-up. Our findings indicated that CPAGN with isolated C3 deposition might have a favorable prognosis after aggressive immunosuppressive treatment. However, the influence of isolated C3 deposition on CPAGN prognosis remains to be clarified.

Efficacy and safety of Shenyankangfu tablets for primary glomerulonephritis: study protocol for a randomized controlled trial.

BACKGROUND: Chronic kidney disease is a common disease. Most chronic kidney diseases evolve from primary glomerulonephritis. Proteinuria is an independent risk factor for the progression of chronic kidney disease. The general consensus is that therapy administered to decrease proteinuria should include steroids and/or immunosuppressants, angiotensin-converting enzyme inhibitors, and angiotensin II receptor blockers. However, the side effects of, and adverse reactions to, these agents reduce the benefits to patients. In addition, the cost of these drugs is relatively high. Therefore, identification of inexpensive and effective drugs to decrease proteinuria is urgently needed. Shenyankangfu tablets have been a widely applied Chinese patent medicine for many years to decrease proteinuria. However, there is a lack of research-derived data regarding the clinical use. Therefore, we designed the present randomized controlled clinical trial to compare the efficacy and safety of Shenyankangfu tablets versus losartan potassium for control of proteinuria in patients with primary glomerulonephritis. METHODS/DESIGN: This study will be a multicenter, prospective, double-blind, double-dummy, randomized controlled clinical trial. We will enroll 720 patients diagnosed with primary glomerulonephritis. The eligible patients will be randomly divided into the following groups at a 1:1:1:1:1 ratio: Shenyankangfu tablets group, losartan potassium 50 mg group, losartan potassium 100 mg group, Shenyankangfu tablets + losartan potassium 50 mg group, and Shenyankangfu tablets + losartan potassium 100 mg group. All groups will be followed up for 48 weeks; follow-up visits will be performed, at weeks 0, 4, 8, 12, 24, 36, and 48. The primary efficacy outcome will be the post-treatment change in the 24-hour proteinuria level, and the secondary efficacy outcomes will be the post-treatment changes in the serum creatinine level, estimated glomerular filtration rate, traditional Chinese medicine syndrome score, and serum albumin level. DISCUSSION: The results of this trial will provide solid data for use in evidence-based medicine with respect to the efficacy and safety of Shenyankangfu tablets for control of proteinuria in patients with primary glomerulonephritis compared to those of losartan potassium. Moreover, we infer that therapy comprising Shenyankangfu tablets + losartan potassium can decrease proteinuria to a larger extent than Shenyankangfu tablets or losartan potassium can alone. TRIAL REGISTRATION: This trial was registered on 12 February 2014 at ClinicalTrials.gov (ID number NCT02063100).

Good response of low-fat/high-protein diet in a patient with chyluria.

UNLABELLED: Chyluria is an inappropriate urinary excretion of chyle that turns the urine milky. A nutritional approach based on low-fat/high-protein content diet associated or not with medium-chain triglyceride (MCT) showed to be an efficient conservative treatment to improve the milky urine appearance in a patient with chyluria. CASE REPORT: A 30-year-old female patient was admitted with chyluria of unknown etiology. An ureteropyeloscopy revealed a single lesion in each kidney, both with linear aspect and measuring 5 mm in extension. These lesions were located close to the renal papillae and were leaking a cloudy and milky fluid. Both lesions were laser cauterized followed by improvement of the milky urine. However, the chyluria relapsed after few months and a low-fat/high-protein content diet with 10 g of soybean oil to meet the requirements essential fatty acids (EFA) and with MCT from coconut oil as alternative to prepare foods was started. Few weeks later the patient returned reporting consistent improvement of the milky urine appearance related with the use of the diet. However since the diet was tasteless and time consuming to prepare, she reported low compliance to diet with MCT and the milky urine relapsed. The MCT was discontinued and the diet with EFA source was maintained with better compliance. Since then the chyluria remains in remission. In conclusion, the dramatic improvement of the milky urine with low-fat/high-protein diet with EFA source observed in our patient demonstrates that this nutritional approach is efficient with fast results to treat chyluria during long term.

Multi-glycoside of Tripterygium wilfordii Hook f. ameliorates prolonged mesangial lesions in experimental progressive glomerulonephritis.

BACKGROUND/AIMS: Multi-glycoside from Tripterygium wilfordii Hook f. (GTW) is used for treatment of progressive glomerulonephritis (GN) in China. We have previously reported the beneficial effects of GTW on acute GN induced by an anti-Thy-1.1 monoclonal antibody (mAb). In the present study, the effect and potential mechanisms of GTW on the chronic irreversible model of GN were investigated. METHODS: Progressive GN was induced in rats by two intravenous injections of anti-Thy-1.1 mAb 1-22-3. Daily oral administration of GTW was started before the second injection of mAb until the day of sacrifice. Ten rats were randomly divided into a control (vehicle-treated) and a GTW-treated group, and sacrificed on day 45 after the first injection of mAb 1-22-3. Proteinuria was determined on days 0, 1, 3, 5, 7, 10, 14, 20, 25, 30, 35, 40, and 45. Blood biochemical parameters, morphological changes of mesangium, glomerular infiltration of macrophage and T lymphocyte, and glomerular mRNA expression of cytokines (TGF-beta, IL-2, and IFN-gamma) were examined from the samples taken at terminal sacrifice. RESULTS: GTW treatment significantly ameliorated proteinuria, renal function, prolonged mesangial lesions and inflammatory cell accumulation in glomerulus. In addition, it significantly reduced the glomerular mRNA expression for TGF-beta, IL-2, and IFN-gamma. CONCLUSION: GTW ameliorates prolonged glomerular lesions presumably through suppression of cytokine production (TGF-beta, IL-2, and IFN-gamma). GTW could be an effective therapeutic agent for treatment of chronic renal diseases.

[Correlation analysis on blood stasis syndrome, clinical features and renal pathology in 174 patients with primary glomerular diseases].

OBJECTIVE: To investigate the relationship between degree of TCM blood-stasis syndrome (BSS) with clinic features and renal pathological type of primary glomerular disease (PGD). METHODS: On-site investigation was adopted, 174 patients with PGD conforming to the inclusive/exclusive criteria were enrolled, and their degree of BSS and deficiency syndrome were scored in 3 days before renal biopsies. The relation of clinical indexes, including age, course of disease, symptoms of deficiency syndrome, 24-h urinary protein excretion (Upro), condition of hypertension and its controlling, glomerular filtrating rate (GFR) based on the predigesting equation of MDRD, and blood levels of uric acid (UA), triglyceride (TG), cholesterol (CHO), hemoglobin (Hb), and albumin (ALB), with the renal pathological type and the BSS score were analyzed. RESULTS: (1) Among the 174 patients, 159 cases (91.38%) were differentiated as BSS, with the degree of moderate in 111 cases and severe in 48 cases; (2) The BSS score was significantly correlated with the level of Upro, CHO, TG, ALB and deficiency syndrome (P < 0.01), but showed insignificant correlation with age, course of disease, grade of the hypertension, and GFR, UA and Hb levels. Multivariate stepwise regression analysis showed that the level of Upro and TG and score of deficiency syndrome had significance for regression equation establishment (P<0.01). (3) Further analysis on renal pathological type in 119 patients of non-nephrotic syndrome showed that the BSS score was insignificantly different among patients with different renal pathological types as the minor/minimal type (3 cases), the focal/segmental glomerular type (72 cases), and the diffuse glomerulonephritis (44 cases, P > 0.05). Further stratified analysis on the 72 cases with focal/segmental lesion showed that BSS score in patients of focal proliferative sclerosing glomerulonephritis were significantly higher than that in those of focal proliferative glomerulonephritis (P < 0.01). CONCLUSION: BSS is a TCM syndrome most commonly seen in patients with primary glomerular disease, BSS score is significantly correlated with the level of Upro, TG and deficiency syndrome score, and exhibits a higher level in patients with focal proliferative glomerulonephritis accompanying glomerulus sclerosis, indicating that the BSS could give certain clues of the renal chronic changes of primary glomerular disease, being one of risk factors in TCM syndrome in the development of renal diseases.

[A crystallographic method in the diagnosis of kidney diseases]. / Kristallograficheskii metod v diagnostike zabolevanii pochek.

Structural aspects of copper chloride crystallization of the urine of patients with pyelonephritis and glomerulonephritis were studied by electron microscopy. It was found that admixtures of urea, creatinine, potassium and, possibly, sodium contained in the urine of patients initiate the formation of copper chloride crystals of different sizes, their shape changes, dendritic and spherolithic crystallization occurs. Results may be used as supplementary differential diagnostic signs of glomerulonephritis and pyelonephritis.