Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors

doi:10.1053/j.ajkd.2003.08.040

American Journal of Kidney Diseases

Volume 42, Issue 6, December 2003, Pages 1154-1163

https://doi.org/10.1053/j.ajkd.2003.08.040 Get rights and content

Abstract

Background: Light chain deposition disease (LCDD) is characterized by the tissue deposition of monotypical immunoglobulin light chains (LCs). The aim of this study was to investigate its clinical characteristics and prognostic factors. Methods: Multicenter study of LCDD with renal and patient survival analyses. Results: Sixty-three cases were studied (age: 58 ± 14.2; males: 63.5%; κ/λ deposition: 68/32%; underlying disorders: multiple myeloma [MM] 65%, lymphoproliferative disorders 3%, idiopathic 32%). Ninety-six percent presented with renal insufficiency (acute, 52%; chronic, 44%), and 84% with proteinuria >1 g/d. During the follow-up, 36 patients reached uremia (incidence rate: 23.7/100 patient-years) and 37 died (17.5/100 patient-years). The factors independently associated with a worse renal prognosis were age (relative risk [RR], 1.05; 95% confidence interval [CI], 1.009 to 1.086) and serum creatinine at presentation (RR, 1.24; 95% CI, 1.02 to 1.5). Those independently associated with a worse patient survival were age (RR, 1.06; 95% CI, 1.03 to 1.1), MM (RR, 2.75; 95% CI, 1.22 to 6.2), and extrarenal LC deposition (RR, 2.24; 95% CI, 1.15 to 4.35). While κ-LC deposition was more frequently associated with nodular sclerosing glomerulopathy, histological parameters were not predictors of renal/patient prognosis. The survival of the uremic patients undergoing dialysis was similar to that of patients not reaching uremia. Conclusion: LCDD is characterized by renal insufficiency with proteinuria and has a severe prognosis. Apart from age, the prognostic factors identified were degree of renal insufficiency at presentation affecting the renal prognosis, underlying hematologic disorder and extrarenal LC deposition affecting the patient prognosis. Dialysis is worth performing in uremic LCDD patients.

Section snippets

Patients and data collection

We retrospectively reviewed the cases of LCDD diagnosed by means of renal histology in 5 nephrological centers in Northern Italy between 1978 and 2002. The cases were analyzed in terms of the pathological and clinical findings at renal biopsy and renal and patient outcome. The information was obtained by means of a chart review and direct contacts with referring physicians.

All of the renal biopsies were processed for light microscopy (LM) and immunofluorescence (IF) according to standard

Patient characteristics at baseline

Sixty-three cases of LCDD were identified, 1 of which was associated with γ-heavy chain deposition. The clinical characteristics at renal biopsy are summarized in Table 1. The mean patient age was 58 ± 14.2 years (range, 28 to 94 years); there were more males than females (63.5% versus 36.5%) and more cases of κ-LC deposition than of λ-LC deposition (68% versus 32%). MM was diagnosed in 65% of the patients and lymphoproliferative disorders (chronic lymphatic leukemia) in 3%; 32% did not meet

Discussion

Our case series showed that LCDD is more frequent in the elderly (from the fifth decade on) and in males. However, it is worth noting the wide age interval (from 28 to 94 years), which indicates that age per se does not exclude a diagnosis of LCDD. There was a prevalence of κ-LC deposition and MM as a hematological disease underlying LC deposition. LCDD was associated with a high prevalence of monoclonal protein in both serum and urine, but 6% of our patients did not have any detectable serum

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Light chain deposition disease (LCDD) is a rare hematologic disorder characterized by non-amyloid monoclonal immunoglobulin light chain deposition in multiple organs. Pulmonary LCDD (PLCDD) is an uncommon manifestation of LCDD usually seen in middle-aged patients presenting with radiologic cystic and nodular findings. We report the case of a 68-year-old female who presented with shortness of breath and atypical chest pain. Chest computerized tomography (CT) scan revealed numerous diffuse but basilar predominant pulmonary cysts and mild bronchiectasis without nodular disease. Given concomitant abnormal renal function and hepatic laboratory indices, she underwent biopsy of both organs confirming the presence of LCDD. Directed chemotherapy was initiated and stabilized renal and hepatic progression, but on follow-up imaging, pulmonary disease appeared worse. While therapeutic options targeting other organ involvement are available, their directed efficacy for progressive lung disease is not well known.
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Original investigation: pathogenesis and treatment of kidney disease and hypertensionLight chain deposition disease with renal involvement: clinical characteristics and prognostic factors

Abstract

Section snippets

Patients and data collection

Patient characteristics at baseline

Discussion

Manifestations of systemic light chain deposition

Am J Med

The patient with insidious chronic renal failure and the patient with the nephrotic syndrome—Two manifestations of a protean and not so rare disease

Nephrol Dial Transplant

Renal disease and patient survival in light chain deposition disease

Clin Nephrol

Long-term follow-up and response to chemotherapy in patients with light-chain deposition disease

Am J Kidney Dis

Renal monoclonal immunoglobulin deposition diseaseThe disease spectrum

J Am Soc Nephrol

Long-term outcome according to renal histological lesions in 118 patients with monoclonal gammopathies

Nephrol Dial Transplant

Plasma cell disordersRecent advances in the biology and treatment

Light-chain deposition diseaseIts relation with AL-type amyloidosis

Kidney Int

Overrepresentation of the VkIV subgroup in light chain deposition disease

Immunol Lett

Monoclonal immunoglobulin deposition diseaseLight chain and light and heavy chain deposition disease and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis

Ann Intern Med

Synthesis of abnormal immunoglobulins in lymphoplasmacytic disorders with visceral light chain deposition

Am J Med

Structure of a monoclonal kappa chain of the VkIV subgroup in the kidney and plasma cells in light chain deposition disease

J Clin Invest

Original investigation: pathogenesis and treatment of kidney disease and hypertension
Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors

Structure of a monoclonal kappa chain of the Vk_IV subgroup in the kidney and plasma cells in light chain deposition disease