scholarly journals Glomerulonephritis with associated hypocomplementemia and crescents: an unusual case of fibrillary glomerulonephritis.

1995 ◽  
Vol 6 (2) ◽  
pp. 171-176
Author(s):  
D B Adey ◽  
B R MacPherson ◽  
G C Groggel
Keyword(s):  
Unusual Case ◽  
Fibrillary Glomerulonephritis ◽  
Pathologic Features ◽  
Immunotactoid Glomerulopathy

Fibrillary glomerulonephritis is an unusual, but not rare cause of glomerulonephritis. Hypocomplementemia in association with fibrillary glomerulonephritis has been reported only once previously. A patient with hypocomplementemia and fibrillary deposits as demonstrated by electronmicroscopy is reported. The clinical and pathologic features of fibrillary glomerulonephritis and immunotactoid glomerulopathy are reviewed.

scholarly journals Fibrilo-Tactoid Glomerulonephritis: A Possible Novel Morphological Variant

2020 ◽  
Vol 10 (3) ◽  
pp. 154-162
Author(s):  
Amaresh Vanga ◽  
Sandeep Magoon ◽  
Jolanta Kowalewska ◽  
Saad Mussarat
Keyword(s):  
Clinical Implications ◽  
Pathological Features ◽  
Hollow Core ◽  
Fibrillary Glomerulonephritis ◽  
Aged Woman ◽  
Immunotactoid Glomerulopathy ◽  
Middle Aged Woman ◽  
Nephrotic Range Proteinuria ◽  
Diagnostic Importance ◽  
Immunotactoid Glomerulonephritis

Fibrillary and immunotactoid glomerulonephritis are infrequent causes of primary nephrotic range proteinuria and are poorly understood. Recent significant developments include the discovery of DNA JB9 antigen in fibrillary glomerulonephritis. Here, we present a case of a middle-aged woman who presented with nephrotic range proteinuria, hematuria, and normal renal function. Renal biopsy revealed fibrils that were randomly arranged on electron microscopy. They were of small size and congo red negative similar to the ones found in fibrillary glomerulonephritis, but were also DNA JB 9 negative, and had a hollow core like in immunotactoid glomerulopathy. Though we try to classify these conditions into either immunotactoid glomerulonephropathy (ITGN) or fibrillary glomerulonephritis (FGN), there are scenarios such as this case where it does not fit into either and is probably an overlap or intermediate variant of these two conditions. Pathological features of these glomerulonephrites are discussed together with their clinical implications, treatment choices, and diagnostic importance.

scholarly journals Fibrillary Glomerulonephritis

2019 ◽  
Vol 14 (12) ◽  
pp. 1741-1750 ◽  
Author(s):  
Nicole K. Andeen ◽  
Megan L. Troxell ◽  
Maziar Riazy ◽  
Rupali S. Avasare ◽  
Jessica Lapasia ◽  
...  
Keyword(s):  
Congo Red ◽  
Primary Outcome ◽  
Multivariable Analysis ◽  
Specific Marker ◽  
Fibrillary Glomerulonephritis ◽  
Pathologic Features ◽  
Highly Sensitive ◽  
Kidney Insufficiency ◽  
Male Sex ◽  
Fibril Diameter

Background and objectivesFibrillary GN has been defined as an immune complex-mediated GN with amyloid-like fibrils larger than amyloid which are IgG positive and Congo red negative. With discovery of DNAJB9 as a highly sensitive and specific marker for fibrillary GN, the specificity of the morphologic criteria for establishing the diagnosis of fibrillary GN has come into question.Design, setting, participants, & measurementsWe sought to (1) determine anatomic characteristics that best define fibrillary GN and (2) identify clinical and pathologic features that predict outcomes.ResultsWe retrospectively reviewed kidney biopsies from patients diagnosed with fibrillary GN or suspected fibrillary GN between 1997 and 2017 (n=266, 65% female, median age 61). Approximately 11% of kidney biopsies had one or more unusual feature including monotypic deposits, Congo red positivity, or unusual fibril diameter. Fibrillary GN as a possible monoclonal gammopathy of renal significance represented <1% of cases. Immunostaining for DNAJB9 confirmed fibrillary GN in 100% of cases diagnosed as fibrillary GN and 79% of atypical cases diagnosed as possible fibrillary GN. At a median time of 24 months (interquartile range, 8–46 months) after biopsy (n=100), 53% of patients reached the combined primary outcome of ESKD or death, 18% had CKD, and 18% had partial remission. On multivariable analysis, male sex (adjusted hazard ratio [aHR], 3.82; 95% confidence interval [95% CI], 1.97 to 7.37) and eGFR were the most significant predictors of primary outcome (aHR of 8.02 if eGFR <30 ml/min per 1.73 m2 [95% CI, 1.85 to 34.75]; aHR of 6.44 if eGFR 30 to <45 ml/min per 1.73 m2 [95% CI, 1.38 to 29.99]). Immunosuppressive therapy with rituximab was significantly associated with stabilization of disease progression.ConclusionsDetection of DNAJB9 is a useful diagnostic tool for diagnosing atypical forms of fibrillary GN. The outcomes for fibrillary GN are poor and progression to ESKD is influenced predominantly by the degree of kidney insufficiency at the time of diagnosis and male sex. Rituximab may help preserve kidney function for select patients with fibrillary GN.PodcastThis article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2019_11_04_CJN03870319.mp3

scholarly journals AN UNUSUAL CASE OF ACUTE HYPOXIC RESPIRATORY FAILURE IN A PATIENT WITH FIBRILLARY GLOMERULONEPHRITIS

CHEST Journal ◽  
2020 ◽  
Vol 158 (4) ◽  
pp. A742-A743 ◽  
Author(s):  
Kushagra Gupta ◽  
Toribiong Uchel ◽  
Shahzad Hussain ◽  
Venkateswara Kollipara ◽  
Domingo Franco-Palacios ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Unusual Case ◽  
Fibrillary Glomerulonephritis ◽  
Hypoxic Respiratory Failure

scholarly journals Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy.

1998 ◽  
Vol 9 (12) ◽  
pp. 2244-2252
Author(s):  
G S Markowitz ◽  
J T Cheng ◽  
R B Colvin ◽  
W M Trebbin ◽  
V D D'Agati
Keyword(s):  
Hepatitis C ◽  
Glomerular Disease ◽  
Hcv Infection ◽  
Not Given ◽  
Fibrillary Glomerulonephritis ◽  
Persistent Proteinuria ◽  
Immunotactoid Glomerulopathy ◽  
Membranous Glomerulopathy ◽  
Cryoglobulinemic Glomerulonephritis

The most common form of glomerular disease seen in association with hepatitis C virus (HCV) infection is membranoproliferative glomerulonephritis, with or without associated cryoglobulinemia. This study examines four cases of fibrillary glomerulonephritis and two cases of immunotactoid glomerulopathy in association with HCV infection. Findings at presentation included proteinuria, renal insufficiency, and hematuria. Renal biopsy revealed a membranoproliferative pattern of glomerular disease in five cases, and a membranous glomerulopathy with mesangial proliferative features in one. On immunofluorescence, all cases stained with IgG and C3. Electron microscopy revealed fibrils of the expected diameter, 16 to 28 nm in fibrillary glomerulonephritis and 33 to 45 nm in immunotactoid glomerulopathy. In only one case were cryoglobulins detected (at low titer and on only one of three assays). Antiviral therapy was not given in any of the six cases. Outcomes were mixed, with progression to renal failure occurring in two patients and persistent proteinuria with stable or improved renal function in three. Follow-up is not available on the sixth case. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have features that overlap with cryoglobulinemic glomerulonephritis. The relatedness of these three entities in a subset of patients with HCV infection suggests a common pathogenic mechanism of glomerular deposition of organized deposits.

scholarly journals An unusual case of a plasma cell neoplasm with an IgG3lambda myeloma and a gamma3 heavy chain disease protein

Blood ◽  
1978 ◽  
Vol 51 (1) ◽  
pp. 85-96 ◽  
Author(s):  
JB Adlersberg ◽  
V Grann ◽  
D Zucker-Franklin ◽  
B Frangione ◽  
EC Franklin
Keyword(s):  
Heavy Chain ◽  
Unusual Case ◽  
Serum Proteins ◽  
Mutational Event ◽  
Myeloma Protein ◽  
Plasma Cell Neoplasm ◽  
Pathologic Features ◽  
Disease Protein ◽  
Probable Origin ◽  
Heavy Chain Disease

Abstract A unique case of gamma3 heavy chain disease with two related serum proteins is reported. One molecule appears to be an IgG3lambda myeloma protein. The second molecule is a dimer of a shortened gamma3 heavy chain that has an unblocked amino terminus and lacks the VH and CH1 domains. Its probable origin as a synthetic product is discussed. The clinical and pathologic features of this patient resemble those of other patients with gamma heavy chain disease more than those of patients with multiple myeloma. It seems likely that the heavy chain disease protein is the result of a mutational event in the malignant clone originally producing the myeloma protein.

scholarly journals Clinical and pathologic features of fibrillary glomerulonephritis

1992 ◽  
Vol 42 (6) ◽  
pp. 1401-1407 ◽  
Author(s):  
Samy S. Iskandar ◽  
Ronald J. Falk ◽  
J. Charles Jennette
Keyword(s):  
Fibrillary Glomerulonephritis ◽  
Pathologic Features

Alpha-Thalassemia Major Presenting in a Term Neonate without Hydrops

2005 ◽  
Vol 8 (6) ◽  
pp. 706-709 ◽  
Author(s):  
Sara E. Monaco ◽  
Mary Davis ◽  
Ay-chyn Huang ◽  
Govind Bhagat ◽  
Rebecca N. Baergen ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Differential Diagnosis ◽  
Clinical Picture ◽  
Blood Smear ◽  
Unusual Case ◽  
Thalassemia Major ◽  
Term Neonate ◽  
Severe Hypoxia ◽  
Alpha Thalassemia ◽  
Pathologic Features

We describe the clinical and pathologic features of an unusual case of alpha-thalassemia major in a patient who survived to term and lived for 9 days. The neonate was nonhydropic and the clinical picture was dominated by severe hypoxia with pulmonary hypertension. The diagnosis was not suspected until postnatal examination of the blood smear, which prompted the performance of hemoglobin electrophoresis and subsequent molecular confirmation. This case illustrates that alpha-thalassemia major should be in the differential diagnosis of hypoxic neonates even in the absence of hydrops.

Classic Adamantinoma with Osteofibrous Dysplasia-like Foci and Secondary Aneurysmal Bone Cyst

2003 ◽  
Vol 6 (2) ◽  
pp. 173-178 ◽  
Author(s):  
Angelica Putnam ◽  
Suzanne Yandow ◽  
Cheryl M. Coffin
Keyword(s):  
Aneurysmal Bone Cyst ◽  
Unusual Case ◽  
Bone Cyst ◽  
Bone Lesion ◽  
Giant Cells ◽  
Multinucleated Giant Cells ◽  
Osteofibrous Dysplasia ◽  
Pathologic Features ◽  
History Of ◽  
Secondary Aneurysmal Bone Cyst

Adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.

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