Fibrillary glomerulonephritis associated with Behçet's syndrome

Fibrillary glomerulonephritis (FGN) is a rare cause of progressive renal dysfunction resulting in fibrillary deposits in the mesangium and/or glomerular basement membrane (GBM). Some case reports have shown FGN in patients with rheumatoid arthritis and other autoimmune diseases. This is the first case report of FGN in a patient with Beh?et's syndrome. The most common renal histological finding in Beh?et's syndrome is secondary amyloidosis. A 46-year-old woman with a 4-year history of Beh?et's syndrome was referred to the nephrology clinic with foamy urine with non-selective proteinuria (urine protein-to-creatinine ratio was 1400 mg protein/g creatinine) and microscopic hematuria. Serum and urine protein electrophoresis showed no evidence of monoclonal gammopathy. A renal biopsy was performed. Light microscopy showed mesangial widening and nodular expansion with hyaline deposits. Immunofluorescence microscopy revealed immunoglobulin M deposits in the mesangium. Congo red staining was negative. Electron microscopy showed fibrillary deposits on the GBM. Pathological findings were consistent with FGN. She had been taking 50 mg azathioprine and 3000 mg mesalazine per day for 4 years due to Beh?et's syndrome, so we did not add any other immunosuppressive agents or corticosteroids. Treatment of this glomerulopathy is not promising. It has been noted that none of the various approaches, including corticosteroid, plasmapheresis, and cytotoxic therapy, improves prognosis.     

Carcinoid tumor and membranous glomerulonephritis: coincidence or malignancy-associated glomerulonephritis?

Membranous glomerulonephritis is known to be associated with malignancies. A 43-year-old man with a history of chronic renal insufficiency secondary to 20-year-old membranous glomerulonephritis was operated on for an infrarenal aneurysm. During surgical intervention, multiple nodular liver lesions were detected. Histologic examination of these lesions showed metastases of a carcinoid tumor. Despite extensive examination, the primary tumor site could not be detected. The patient remained asymptomatic 3 years postoperatively without any treatment for carcinoid tumor. This clinical report is the second case of a membranous glomerulonephritis associated with a carcinoid tumor. Whether the association is merely a coincidence or a real malignancy-related glomerulopathy remains unclear. Because survival of 23 years after the onset of symptoms of carcinoid tumor has occurred, it is possible that our patient already had an asymptomatic carcinoid tumor at the time the diagnosis of membranous glomerulonephritis was made. Comparison with other paraneoplastic glomerulonephritis as well as diagnosis of a carcinoid tumor in renal insufficiency are discussed.     

Long-term follow-up of atypical membranoproliferative glomerulonephritis: are steroids indicated?

Atypical membranoproliferative glomerulonephritis (MPGN) has been reported to have a good prognosis when treated with corticosteroids. However, this recommendation is based on uncontrolled trials and is associated with many complications. The purpose of our study is to determine whether steroid therapy is indicated for atypical MPGN. The cases of seven patients with atypical MPGN are reported in this study. Urinary abnormalities of five of them were detected by urine screening at school, of two because of macrohematuria. Hypocomplementemia was noted in six patients. All but one patient were treated without corticosteroids, and five with angiotensin-converting enzyme inhibitors (ACEI) and/or the Chinese herbal medicine Sairei-to (TJ-114). One patient recovered spontaneously from proteinuria and was therefore not treated, and one who developed severe proteinuria during observation was treated with corticosteroids. After an average follow-up period of 10.0 years, five patients showed normal urinary findings, one had hematuria and one proteinuria. At the most recent follow-up, the renal function of all patients remained within the normal range, and serum C3 had returned to normal levels in five out of six. These findings suggest that the indication of steroid therapy for atypical MPGN should be re-examined, since most of the patients with atypical MPGN seem to have an excellent prognosis without treatment with corticosteroids.     

Glomerular involution in children with frequently relapsing minimal change nephrotic syndrome: An unrecognized form of glomerulosclerosis?

Global glomerulosclerosis can be divided in the vascular (obsolescent) type and the glomerulopathic (solidified) type. In biopsies from children with recurrent nephrotic syndrome owing to minimal change nephropathy (MCN), we noticed small, globally sclerosed glomeruli that appeared to be distinct from global glomerulosclerosis. These small sclerosed glomeruli are best described as involuted glomeruli. We have characterized these involuted glomeruli in detail. We studied biopsies of 18 children (11 male, 7 female) with frequently relapsing MCN and evaluated possible explanatory variables. The involuted glomeruli can be differentiated from the other types of global glomerulosclerosis. Most notable is the presence of vital podocytes and parietal epithelial cells, which have retained their staining characteristics, in between the matrix, and the absence of periglomerular and tubulo-interstitial fibrosis. We observed involuted glomeruli in 12 out of 18 biopsies; the median percentage of involuted glomeruli was 6% (range 0-33%). The percentage of involuted glomeruli correlated with age at renal biopsy and the interval between onset of disease and time of renal biopsy, but not with gender, age at onset of disease, or prednisone dose. Multivariate analysis revealed that the interval between onset of disease and time of renal biopsy was the only independent predictor. In conclusion, glomerular involution is a special form of global glomerulosclerosis. The absence of periglomerular and tubulo-interstitial fibrosis suggests a different pathogenesis. Glomerular involution is a slow process. The clinical data suggest that involution is related to the duration of the disease process.     

Peripheral aneurysms and arteriomegaly: Is there a familial pattern?

Purpose: Studies have shown that 11% to 18% of patients with an abdominal aortic aneurysm (AAA) have a first-degree relative with an AAA. A familial pattern among patients with peripheral arterial aneurysms and arteriomegaly has not been reported. The objective of this study was to examine familial patterns among patients with peripheral arterial aneurysm and arteriomegaly and compare them with patterns among patients with AAA. Methods: Pedigrees were constructed for first-degree relatives of patients who received the diagnosis of peripheral arterial aneurysm, arteriomegaly, or AAA from 1988 through 1996. The presence of aneurysms and risk factors was confirmed for patients and relatives by means of telephone interviews and review of hospital and physician records. Results: Seven hundred three first-degree relatives older than 50 years were contacted for 140 probands with peripheral arterial aneurysm, AAA, or arteriomegaly. There were differences in risk factors for hernia and diabetes mellitus among the probands with peripheral arterial aneurysm, AAA, or arteriomegaly but none for relatives. Patients with peripheral arterial aneurysm (n = 40) had a 10% (4/40) familial incidence rate of an aneurysm, patients with AAA (n = 86) had a 22% (19/86) familial incidence rate, and patients with arteriomegaly (n = 14) had a 36% (5/14) familial incidence rate. AAA (24/28, or 86%) was the aneurysm diagnosed most commonly among first-degree relatives. Most aneurysms (85%) occurred among men. Conclusion: There appears to be a gradation of familial patterns from peripheral arterial aneurysm to AAA to arteriomegaly among patients with degenerative aneurysmal disease, and there appears to be a predominance among men. Relatives of patients with any of the 3 lesions—peripheral arterial aneurysm, AAA, arteriomegaly—most frequently have AAA. Relatives of patients with AAA, peripheral arterial aneurysm, or arteriomegaly may be screened by means of a physical examination for peripheral aneurysmal disease. Screening by means of ultrasound examination of the aorta should be limited to first-degree relatives of patients with aortic aneurysms or arteriomegaly. (J Vasc Surg 1998;28:599-605.)     

Pathology and immunology of lupus glomerulonephritis: can we bridge the two?

Aberrant immune responses underwrite lupus glomerulonephritis and may contribute to glomerular cell proliferation and inflammation. Recent studies provide evidence that apoptotic immune cells may initiate immune events leading to tissue damage. Nucleosomes within apoptotic particles are recognized by B cells and other antigen presenting cells and represent the most likely inciting antigen for autoantibody production. Some of these antibodies are nephritogenic depending on fine structural composition and antigen recognition in the circulation or on renal cells. Deficient complement components contribute to reduced clearance of circulating and native kidney apoptotic cells. This review summarizes current concepts in lupus immune pathogenesis and attempts to bridge immunology to pathology of lupus glomerulonephritis.     

The new consent form: is it any better?

BACKGROUND: The UK Department of Health (DoH) introduced a new consent form into the National Health Service (NHS) in April 2002 following the Bristol Royal Infirmary Inquiry. AIM: To compare the efficacy of the new consent form with the old on the quality of consent. METHODS: A questionnaire consisting of 11 questions was distributed to two groups of 100 patients before and after the introduction of the new consent form in the pre-assessment clinic at Glenfield Hospital, Leicester. RESULTS: Of the 11 questions, there were four significant differences that favoured the new consent form. These were: (i) success and benefits of the operation (old, 81%; new, 97%, P < 0.001; 95% CI, 7.3-24.4%); (ii) information that patients received about the operation from the doctor (old, 34%; new, 93%; P < 0.001; 95% CI, 46.7%-68.9%) and nurse (old, 21%; new, 67%; P < 0.001; 95% CI, 33.3-58.3%) in the pre-assessment clinic; (iii) postoperative recovery (old, 56%; new, 96%; P < 0.001; 95% CI, 30.0%-51.2%); and (iv) ability to list potential complications that could arise from the operation (old, 61%; new, 97%; P < 0.001; 95% CI, 26.4-52.6%). Despite the above differences, an overall assessment involving all questions failed to show a significant improvement with the new consent form (old, 57%; new, 67%; P = 0.264;, 95% CI, -35.6% to 12.6%). DISCUSSION AND CONCLUSIONS: The new consent form resulted in improvement in some, but not all, aspects of consent and no question reached the ideal standard of 100%. We suggest a formatted consent form for procedures in conjunction with additional information.     

Acute post-streptococcal glomerulonephritis in a 14-month-old boy: why is this uncommon?

Acute post-streptococcal glomerulonephritis (APSGN) is rare in children under 2 years of age. This is related in part to the disease patterns of group A streptococcus (GAS) and in part to impaired immunogenicity in infants. We report the case of a 14-month-old child with APSGN following GAS pharyngitis. This case illustrates that APSGN needs to be considered in the evaluation of both gross and microscopic hematuria in this age group. We review the literature of both GAS and APSGN and discuss the pathogenesis and epidemiologic reasons for this association.     

MPO-ANCA–positive crescentic glomerulonephritis: A distinct entity of scleroderma renal disease?

Scleroderma renal crisis is characterized by intimal thickening of the afferent glomerular arterioles resulting in hypertension and fibrinoid necrosis of the capillary tuff. We report a 67-year-old man with long-standing systemic sclerosis who developed normotensive progressive renal failure, proteinuria, and a nephritic urinary sediment with serum myeloperoxidase-antineutrophil cytoplasmatic antibodies (MPO-ANCA). Renal biopsy showed pauci-immune crescentic glomerulonephritis but none of the typical vascular changes of scleroderma renal crisis. Because comparable cases have recently been reported from Japan, normotensive MPO-ANCA—positive crescentic glomerulonephritis may form an entity of progressive renal failure in scleroderma.     

Renal granuloma and immunoglobulin M-complex glomerulonephritis: a case of common variable immunodeficiency?

Common variable immunodeficiency (CVID) is characterized by reduced serum immunoglobulin levels and recurrent bacterial infections. Granulomatous infiltrations are occasionally found in the lymphoid or solid organs of affected patients, but renal involvement is rare. We present a case of possible CVID with interstitial noncaseating granuloma and immunoglobulin (IgM)-complex glomerulonephritis with a membranoproliferative pattern and with a favorable response to corticosteroids, intravenously administered immunoglobulins (IVIGs) and rituximab. CVID must be included in the differential diagnosis of renal granuloma and should be differentiated from sarcoidosis to ensure appropriate therapy. The authors declare that they had no financial support.