Paraprotein-related kidney disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ali Poyan Mehr, M.D. [2]

Synonyms and keywords: monoclonal gammopathy of renal significance; MGRS; paraproteinemia

Overview

Paraproteinemia refers to the presence of abnormal protein or protein fragments in the blood. The proteins referred to are immunoglobulins (or fragments of), and are of monoclonal origin (M-spike). Under certain circumstances these circulating monoclonal immunoglobulins are deposited in the renal tissues and can lead to various forms of renal disease.

Historical Perspective

In 1848, Henry Bence Jones (http://rstl.royalsocietypublishing.org/content/138/55) reported on the discovery of an "new substance" in the urine of an ill patient. Over the course of the history, this protein was termed "Bence Jones" and found to be associated with "myeloma". The term "myeloma kidney" was used first by Alfred von Decastello in 1909.

By 1939 the abnormal band described in serum electrophoresis of myeloma patients as "M-spike", was found to be due to immuneglobulin proteins.

Subsequent studies were able to further characterize these abnormal immunoglobulins and define as monoclonal kappa or lambda light chains, and confirm Bence Jones proteins to be the same as the circulating monoclonal immuneglobulins (para-proteins). (http://medcraveonline.com/UNOAJ/UNOAJ-03-00078.pdf)

Classification

• Paraprotein-related kidney disease may be classified according to the type of renal involvement and the underlying hematologic disorder into at least 10 various subtypes:

• AL Amyloidosis (either due to lambda or kappa light chains)
• Cryoglobulinemic GN, Waldenstrom type
• Cryoglobulinemic GN, Multiple Myeloma
• Cryglobulinemia type I: Ideopathic
• Immunotactoid Glomerulopathy
• Fibrillary Glomerulopathy
• Light chain deposition disease
• Heavy chain deposition disease
• Mixed light and heavy chain deposition disease
• Proliferative GN with monoclonal Ig
• Cast nephropathy

Pathophysiology

• Here, the pathophysiology of paraprotein-related kidney disease will be discussed. For the pathophysiology of clonal B-cell disorders, including multiple myeloma, please refer to the corresponding chapters.

The type of kidney involvement highly depends on the specific characteristics of the paraprotein.

• AL Amyloidosis (either due to lambda or kappa light chains)

AL amyloidosis is caused by the deposition of Amyloid deposits in extracellular spaces. These deposits may infiltrate the glomerular basement membrane (GMB) or may be localized on both its sides. Advanced amyloid produces a nonproliferative, noninflammatory glomerulopathy and enlargement of the kidney. The most frequent and severe amyloidosis affecting the kidney is Immunoglobulin-Associated amyloidosis (AL amyloidosis)

• Cryoglobulinemic GN, Waldenstrom type
• Cryoglobulinemic GN, Multiple Myeloma
• Cryglobulinemia type I: Ideopathic
• Immunotactoid Glomerulopathy
• Fibrillary Glomerulopathy
• Light chain deposition disease
• Heavy chain deposition disease
• Monoclonal immunoglobulin deposition disease
• Proliferative GN with monoclonal Ig
• Cast nephropathy

Causes

• [Disease name] may be caused by either [cause1], [cause2], or [cause3].
• [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
• There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].