[show abstract][hide abstract] ABSTRACT: Renal organized or structured deposits are much less frequent than those with usual type immunocomplex deposits and are encountered in a wide variety of primary and systemic disorders. It has been suggested that immunoglobulins (Igs) are responsible for organized deposits. We report 5 cases who have been diagnosed and treated in our hospital. Patients were aged 52 to 72 years, three of them were males and had variable degree of renal function, from normal serum creatinine to uraemia. Proteinuria was detected in all patients while monoclonal component was present only in the serum of one subject. Ultrastructural analysis of renal specimens revealed organized deposits. Diagnoses that were made are the following: membranoproliferative glomerulonephritis with finger print, immunotactoid glomerulopathy, membranoproliferative glomerulonephritis with arched deposits, primary amyloidosis and light chain deposition disease. In systemic disorders ultrastructural pathology could be particularly valuable for correct deposits classification, precise localization and pattern of deposition of Igs.
[show abstract][hide abstract] ABSTRACT: Discussion The clinical picture was suggestive of cryoglobulinaemia although cryoglobulins were detected only once; in fact there is generally no relationship between the severity of vasculitic manifestations and serum levels of cryoglobulins or complements (1). In our case the diagnosis was mainly based on 'Meltzer's triad' of palpable purpura, arthral- gias and myalgias which is generally seen in essential, viral or connective tissue-associated cryoglobulinaemia. Moreover, a malignant tumour of the uterus was discov- ered a few months after the onset of renal disease; there- fore, a link between the two conditions could be hy- pothesized. Glomerulopathies with organized deposits are much less frequent than those with usual-type immune- complex deposits and are typically described in amy- loidosis, cryoglobulinaemic glomerulonephritis, fibrillary glomerulonephritis, immunotactoid glomerulopahty, col- lagenofibrotic glomerulopathy and fibronectin glomeru- lopathy (2). The generic term 'glomerular deposition dis- ease' has been proposed by pathologists (3). To the best of our knowledge this is the first time that comma-shaped electron-dense deposits in the mesangium, subepithelial and subendothelial spaces have been reported. Moreover, Ronco and Aucouturier (4) classified cryoglobulinaemia kidney as a microtubular organized deposit in their patho- logic classification of diseases with tissue depositions or precipitation of monoclonal Ig-related materials. We can- not exclude that the peculiar shape and diffuse distribution that we found could be related to the subsequent diagnosis of malignant uterine neoplasm.
[show abstract][hide abstract] ABSTRACT: Light chain deposition disease (LCDD) can involve the heart and cause severe heart failure. Cardiac involvement is usually described in the advanced stages of the disease. We report the case of a woman in whom restrictive cardiomyopathy due to LCDD presented with paroxysmal atrial fibrillation.
A 55-year-old woman was admitted to our emergency department because of palpitations. In a recent blood test, serum creatinine was 1.4 mg/dl. She was found to have high blood pressure, left ventricular hypertrophy and paroxysmal atrial fibrillation. An ACE-inhibitor was prescribed but her renal function rapidly worsened and she was admitted to our nephrology unit. On admission serum creatinine was 9.4 mg/dl, potassium 6.8 mmol/l, haemoglobin 7.7 g/dl, N-terminal pro-brain natriuretic peptide 29894 pg/ml. A central venous catheter was inserted and haemodialysis was started. She underwent a renal biopsy which showed kappa LCDD. Bone marrow aspiration and bone biopsy demonstrated kappa light chain multiple myeloma. Echocardiographic findings were consistent with restrictive cardiomyopathy. Thalidomide and dexamethasone were prescribed, and a peritoneal catheter was inserted. Peritoneal dialysis has now been performed for 15 months without complications.
Despite the predominant tubular deposition of kappa light chain, in our patient the first clinical manifestation of LCDD was cardiac disease manifesting as atrial fibrillation and the correct diagnosis was delayed. The clinical management initially addressed the cardiovascular symptoms without paying sufficient attention to the pre-existing slight increase in our patient's serum creatinine. However cardiac involvement is a quite uncommon presentation of LCDD, and this unusual case suggests that the onset of acute arrhythmias associated with restrictive cardiomyopathy and impaired renal function might be related to LCDD.