Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the past 10 years, immunotactoid glomerulopathy has become recognized with increasing frequency. The lesion is characterized histologically by highly organized ultrastructural deposits that appear to be composed of immunoglobulin and complement and are negative for amyloid by Congo red stain. Clinically and/or serologically, patients have no evidence of cryoglobulinemia, amyloidosis, systemic lupus erythematosus, or a paraproteinemia, disorders associated with glomerular deposits, which also have a highly organized tactoidal or fibrillar characteristic. Immunotactoid glomerulopathy does not appear to be a multisystemic disease process and thus may represent a primary glomerulopathy. Patients with immunotactoid glomerulopathy present with proteinuria (nephrotic range in more than 60%) and over half of the patients have hypertension, hematuria, and renal insufficiency. Progression to end stage renal disease has occurred in more than 40% of patients reported to date. The experience in treating this disorder using prednisone and/or immunosuppression is limited and has not been impressive. Four patients have successfully undergone renal transplantation, but proteinuria recurred in two and was associated with the recurrence of immunotactoid glomerulopathy in the renal allograft. Although we have gained insight into the clinical course and histopathology of this disorder over the past few years, we still know little about its pathogenesis, an area for further research.
 ...
PMID:Immunotactoid glomerulopathy. 199 64

Findings in 27 patients with typical skin lesions of urticarial vasculitis (UV) who were seen at a connective tissue disease clinic over a 5-year period (1986 to 1990) are reviewed. The majority suffered from systemic lupus erythematosus (SLE) or from "lupus-like" disease (18 patients), 1 from "mixed" connective tissue disease (MCTD), and 5 from primary UV. All of the latter patients had normal serum complement levels (normocomplementemic urticarial vasculitic syndrome; NUVS). No patients with hypocomplementemic UV were encountered. Two patients suffered from necrotizing vasculitis (polyarteritis nodosa, Wegener's granulomatosis); one had a C1-esterase inhibitor deficiency and also demonstrated an immunoglobulin G paraproteinemia. Angioedema occurred in many patients and could not be used as a differential diagnostic feature. The course of the illness was chronic in most patients, lasting for up to 23 years, and the response to therapy was unpredictable, erratic, and unsustained. The use of intravenous "pulse" methylprednisolone, cyclophosphamide, or high-dose oral steroids helped selected patients. Colchicine was dramatically effective in one patient with NUVS of 15 years duration. Azathioprine was not beneficial. None of the five patients with NUVS suffered from severe systemic involvement or renal disease, confirming observations by others that this form of UV represents a milder example of the condition.
 ...
PMID:Urticarial vasculitis in a connective tissue disease clinic: patterns, presentations, and treatment. 206 75

We report a 49-year-old woman who developed lobular glomerulonephritis with prevalent deposition of material positive for IgG, C1q and lambda light chain, but which was not stained by Congo red. Glomeruli revealed massive electron-dense deposits with a microlamellar structure in the mesangial matrix and peripheral capillary loops. Clinically, the patient had nephrotic syndrome, microhematuria and hypertension. No Bence-Jones protein or cryoglobulin was found in the urine or serum. Anti-DNA antibody was positive, but systemic lupus erythematosus (SLE) was ruled out by repeated serological examinations. Immunoelectrophoresis of blood and urine revealed increased IgG-lambda paraprotein, but no free light chains were found. We reviewed 54 cases reported in the literature, which showed organized crystalline structures on ultrastructural examination, but were unassociated with amyloidosis, SLE, cryoglobulinemia or multiple myeloma. The present patient is the first reported to have exhibited a combination of glomerulonephritis with organized deposits, monoclonal IgG lambda paraproteinemia, and the presence of anti-DNA antibody.
 ...
PMID:Microlamellar structures in lobular glomerulonephritis associated with monoclonal IgG lambda paraproteinemia. A case report and review of the literature. 212 87

A review is presented on the use of continuous ambulatory peritoneal dialysis (CAPD) in some systemic diseases with renal involvement to the stage of terminal renal failure: progressive systemic sclerosis, systemic lupus erythematosus, paraproteinemias and systemic amyloidosis. CAPD provides a suitable means of treatment of end-stage renal failure complicating systemic diseases both in terms of control of uremia and quality of life, being the treatment of choice in selected patients.
 ...
PMID:CAPD and systemic diseases. 305 63

The clinical and laboratory findings in seven female patients with primary autoimmune diseases, one female patient with lymphoplasmacytoid (LP) immunocytoma and IgM paraproteinemia, and two male patients with multiple myeloma are described. The common denominator in all patients was a lupus anticoagulant or a closely related coagulation disorder. Recurrent thrombosis was observed in six patients with autoimmune diseases and in two patients with malignant monoclonal gammopathies. Other clinical manifestations included cerebral disorders (four patients with autoimmune disease/two patients with monoclonal gammopathy), repeated obstetric complications (6/1), asymptomatic valvular heart disease (6/1), renal dysfunction (6/2), hepatic involvement (2/2), and arthropathy (2/0). Laboratory investigations revealed a biologic false-positive serological test for syphilis in six patients with autoimmune disease and one with monoclonal gammopathy, antinuclear antibodies (4/0), antibodies against DNA (4/1), and a positive direct Coombs test (3/1) which was accompanied by hemolytic anemia in two patients (1/1). Additionally slight leukocytopenia (2/1) and thrombocytopenia (6/2) were observed; abnormal bleeding was only seen in one patient with severe thrombocytopenia. Other complications characteristic of LP immunocytoma or multiple myeloma were missing. The obvious similarities between the patients with autoimmune diseases and the patients with malignant monoclonal gammopathies suggest analogous pathogenetic mechanisms.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Lupus anticoagulant associated syndrome in benign and malignant systemic disease--analysis of ten observations. 311 94

We present 11 patients with immunotactoid glomerulopathy, a new syndrome characterized clinically by proteinuria (11/11), microscopic hematuria (9/11) and hypertension (9/11). The patients consisted of six females and five males, aged 25 to 59 years (mean, 44.6). Proteinuria was the presenting feature and the reason for renal biopsy in all patients. The diagnosis of immunotactoid glomerulopathy was established at renal biopsy by the presence of glomerular extracellular microtubules composed of immune reactants. All the biopsies studied by immunofluorescence (10 cases) had glomerular deposits of IgG and C3. In three biopsies studied with IgG subclass specific antisera, only one patient had monoclonal immunoglobulin deposits (IgG3 kappa). In six cases the glomerular deposits were analyzed for light chains. In three the deposits contained kappa only, and three consisted of both kappa and lambda. In two cases the immune aggregates were confined to the mesangium, and in the remaining eight cases, the deposits were present in the mesangium and the glomerular basement membranes. Electron-dense deposits composed of microtubules were present in the same distribution within the glomerulus as the immune reactants. The microtubules had a uniform diameter in each biopsy, but they varied in size from case to case. They were approximately the same size in eight cases (mean, 22.3 +/- 3 [SD] nm). Three cases had much larger microtubules: 34.2 nm, 35.4 nm, and 48.9 nm in diameter. Although the 22.3-nm microtubules resembled amyloid in their appearance, glomerular distribution and random orientation in the tissue, they were more than twice the diameter of amyloid (8.9 nm), and Congo red and thioflavin T stains for amyloid were negative. Similar microtubular structures have been described in patients with cryoglobulinemia, SLE and paraproteinemia, but these diseases were excluded in our patients on clinical, serologic and in some cases histologic grounds. More important, none of our patients had clinical or histochemical evidence of amyloidosis, an entity which may be confused with immunotactoid glomerulopathy on a morphologic basis. Follow-up, from 22 to 94 months (mean, 52.6) was obtained in all 11 patients, and 2 clinical courses were noted. Six patients had progressive deterioration of renal function, with five requiring dialysis. This group had severe hypertension (4/6) and nephrotic-range proteinuria (5/6) at some point in their course. The remaining five patients with stable renal function had proteinuria of less than 2.0 g/24 hr in most cases (4/5), and none had severe hypertension. This dichotomy correlated with the distribution of immunotactoids.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Immunotactoid glomerulopathy. 401 May

Increasing numbers of patients are being recognized with neurological abnormalities associated with the immunochemical changes of plasma cell disease. To illustrate the wide spectrum of clinical disorders that can be found, I discuss in detail 5 patients: 2 with neuropathy, 3 with amyotrophic lateral sclerosis (ALS), all of whom had serum monoclonal paraproteinemia. In addition, I report in tabular form 6 patients with paraproteinemia and the following clinical presentations: 1) systemic lupus with polyneuropathy and severe cerebritis, 2) myasthenia gravis with thymoma, 3) polymyositis, 4) polymyositis, arthritis and Grave's disease, 5) relapsing polyneuritis (one of the original patients diagnosed by Austin) and 6) ALS, dystonia and parkinsonism. Major improvements in clinical condition occurred sometimes, but not always, coincident with reductions in the levels of the paraprotein using prednisone, cyclophosphamide, chlorambucil and plasma exchange treatments even in some of the patients who had the clinical appearance of ALS. Patients with neuromuscular diseases should be routinely screened with serum immunoelectrophoresis for monoclonal gammopathy. If a monoclonal gammopathy is found and if the disease is serious, then those patients should be treated as if they had an autoimmune disorder.
 ...
PMID:Neuropathy and motor neuron syndromes associated with plasma cell disease. 647 86

A 9-year-old girl presented with mild proteinuria and microscopic hematuria. Renal biopsy disclosed highly organized fibrillary deposits in glomerular mesangial areas by ultrastructural examination. The microfibrils were 10-15 nm in diameter, up to 1,200 nm in length and in parallel array. They did not have a microtubular appearance. The diameter of the microfibrils was larger than that of amyloid fibrils. Congo red and crystal violet stains were negative. There was no clinical or serologic evidence of paraproteinemia, cryoglobulinemia, light chain disease or systemic lupus erythematosus. To date, most of the reported cases of fibrillary glomerulonephritis have occurred in adults; this disorder is extremely rare in children. We believe that this glomerulopathy can occur in the pediatric age group and should be considered in renal biopsy diagnoses.
 ...
PMID:Fibrillary glomerulonephritis in a 9-year-old girl. 789 2

Immunotactoid glomerulopathy is characterized by the ultrastructural finding of fibrillary or microtubular deposits in patients without systemic diseases such as SLE, diabetes, paraproteinemias, cryoglobulinemia, or amyloidosis. These deposits correspond in most (but not all) cases to immunoglobulin and complement deposits as shown by immunohistochemical techniques. Different light microscopic patterns (mesangioproliferative, membranous, membranoproliferative, and crescentic) have been reported. Clinical presenting feature is characterized by proteinuria (often of nephrotic range), hematuria, and hypertension in most cases. Chronic renal failure requiring hemodialysis or transplantation is described in more than half the patients. Pathogenesis has not yet been elucidated and only some speculative hypotheses have so far been suggested. At present there is no clear evidence that we are dealing with a new pathologic entity, but larger series must be collected and studied in order to find a correct taxonomic collocation of this glomerulopathy.
 ...
PMID:Immunotactoid glomerulopathy (ITGP): a not fully defined clinicopathologic entity. 851 98

We report a 51-year-old man diagnosed as having immunotactoid glomerulopathy (IT) who achieved partial remission after approximately 1 year of a low-dose prednisolone regimen. On admission, he was noted to show proteinuria, hypoproteinemia, and hypocomplementemia. On electron microscopy of the renal biopsy specimen, the mesangial and subendothelial areas were expanded because of the electron-dense deposits, which were represented by mostly straight and nonbranching hollow microtubule structures. The microtubular width was on average 22.0 nm. Clinical and histological findings did not support the diagnosis of amyloidosis, cryoglobulinemic glomerulonephritis, systemic lupus erythematosus, or paraproteinemias. Under treatment with oral prednisolone, 4 months later, the patient's serum albumin level increased from its lowest level of 2.3 to 3.6 g/dL, and CH50 from the lowest level of less than 6.3 to 32.4 U/mL. A 24-hour collection of urine showed that the protein had decreased from its highest level of 3.9 g to 2.0 g. This case suggests the effectiveness of long-term, low-dose prednisolone therapy for IT.
 ...
PMID:Immunotactoid glomerulopathy: report of a case. 921 17


1 2 Next >>