Case of membranoproliferative glomerulonephritis due to essential cryoglobulinemia without hepatitis C virus infection
Takafumi OkuraMasanori JotokuKen‐ichi MiyoshiDaijiro EnomotoMie KurataJun IritaTomoaki NagaoTomoaki OhtsukaJitsuo Higaki
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Abstract:
An 81-year-old man was admitted to our hospital because of leg edema. Serological studies for anti-neutrophil cytoplasmic antibody (ANCA), anti-double stranded DNA antibodies, and antibodies to hepatitis C and B were negative. Severe hypocomplementemia was present and a cryoglobulin was detected with serum immunoelectrophoresis being positive for the immunoglobulin (Ig)M kappa type. The cryoglobulin was characterized by immunoelectrophoresis which showed that the protein was composed of polyclonal IgG and kappa-type monoclonal IgM. A diagnosis of essential type II cryoglobulinemia was made and the patient underwent a renal biopsy. The renal biopsy revealed endocapillary and mesangial cell proliferation with increased matrix. The resultant lobular appearance of the glomerulus and double contours of the basement membrane were indicative of membranoproliferative glomerulonephritis (MPGN). Immunofluorescence studies demonstrated granular staining in the capillary wall for IgG, IgA, IgM and C4 with little C3 deposition but no C1q. He was finally diagnosed with MPGN due to mixed cryoglobulinemia type II. MPGN with essential cryoglobulinemia type II without evidence of hepatitis C virus infection, like that found in the present case, is very rare.Keywords:
Membranoproliferative glomerulonephritis
Cryoglobulins
Hepatitis C
Immunoelectrophoresis
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The technique of cryofiltration has been applied in the treatment of cryoglobulinemia. Good clinical improvement has been demonstrated accompanied by decreases of cryoglobulin levels. Three treatments in a week produced transient improvement of clinical symptoms and reduction of cryoglobulin levels. Follow-up treatments performed at adequate frequency and volume processed maintained relief of symptoms and serologic improvement. The cryofilter demonstrates good selectivity for cryoglobulin removal in cryogel, confirmed by electrophoretic analysis of both the cryogel filtrate from the cryofilter used clinically. Plasma cryofiltration appears to be a safe alternative to plasma exchange, requiring no replacement fluids.
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We present a case of type 1 cryoglobulinemia with an underlying hematological malignancy. Unusually, the entire unifying diagnosis was made on the basis of the renal biopsy. Initially, serum cryoglobulin was not identified; repeat samples were positive. It is important to note that there is a significant false-negative rate with laboratory testing for cryoglobulin, due, at least in part, to the specific conditions required for collection and processing. With that in mind, in all cases with strong clinical or histological evidence of cryoglobulin but negative testing, carefully repeated samples should be sent.
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Background
Systemic lupus erythematosus (SLE), as the prototype of systemic autoimmune diseases, has a wide array of clinical manifestations. An association between cryoglobulinemia and certain clinical and immunological features of SLE has been proposed, and there are various physiopathologic pathways that could be involved in this relationship. Although the presence of cryoglobulinemia in SLE patients may be related to specific disease features and could even have prognostic value, this association has not been addressed thoroughly.Objectives
To describe clinical and immunological characteristics in SLE patients with cryoglobulinemia.Methods
We performed a retrospective, case-control study, in which we included all patients with a cryoglobulin determination between January 2005 and December 2016 in a third level referral centre in Mexico City. Patients with SLE and a positive cryoglobulin test (cryocrit ≥1%) were included in the case group, whereas SLE patients with a negative cryoglobulin determination were considered controls. We studied demographic, clinical and immunological characteristics at the time of the positive cryoglobulin result, as well as three months earlier, and 6 and 12 months later.Results
Thirty-six SLE patients had a cryoglobulin determination throughout the study period. Ten patients had cryoglobulin levels≥1% and were included in the case group, whereas 26 patients with a negative determination were included as controls. Mean age was 37.7±18.3 in cases and 41.7±19.3 in controls. 70% of cases and 88.5% of controls were women. Among subjects with cryoglobulinemia, the cryocrit was 1% in 9 patients, and 3% in one. Regarding clinical and immunological characteristics, a positive lupus anticoagulant and a history of vasculitis were more frequent in patients with serum cryoglobulins (p=0.004 and 0.04, respectively). At the time of the cryoglobulin measurement, patients in the case group had lower levels of C3 and C4 (p=0.026 and p=0.003, respectively), and serum albumin (p=0.028). They also had a higher prevalence of serositis (p=0.021) and peripheral oedema (p=0.034), as well as a higher SLICC Damage Index score (p=0.014) than controls. Regarding follow-up, patients in the case group had a higher SLEDAI score after six and twelve months (p=0.009 and 0.034, respectively). Also, after 12 months they had a higher prevalence of renal activity (p=0.004) and lower C4 levels (p=0.001). Among patients with renal activity, 20% of cases and 55% of controls had achieved complete remission after 12 months.Conclusions
Serum cryoglobulins in SLE patients were associated with positive lupus anticoagulant and hypocomplementemia. Cryoglobulinemia was also associated with specific disease manifestations, such as serositis and vasculitis, and with damage accrual. At follow-up, patients with cryoglobulinemia had a higher prevalence of renal activity, as well as an increased disease activity overall. Whether cryoglobulins could be used as a biomarker for renal activity or worse renal prognosis remains to be determined, and larger prospective studies will be needed to address this possibility.Disclosure of Interest
None declaredCryoglobulins
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Cryoglobulinemia, a condition in which a protein or group of proteins in the blood precipitate on exposure to cold, may be associated with retinal vascular changes. This paper reviews the present knowledge of cryoglobulinemia and presents the eye findings which were present in one of three patients with cryoglobulinemia seen at the Veterans Administration Hospital in Philadelphia. In 1933, Wintrobe and Buell described a cold-precipitable globulin. Others repeated this observation, but not until 1947 was the term cryoglobulin used.4,7,13,16,22,49Lerner and Watson, in 1947, while studying the blood serums of 121 patients suffering from various diseases, found that there was a spontaneous precipitation of protein in 31 cases when the serum was cooled to 4 C.6They proposed the term cryoglobulin to describe the protein or group of proteins which have the characteristic of precipitation, or "gelifying," in the cold. The precipitated protein, which they regarded as a
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Pseudo-leukocytosis was found in a patient with essential cryoglobulinemia when leukocyte (WBC) counts were determined using a Model S Coulter Counter. The spurious leukocytosis was confirmed in additional patients with cryoglobulinemia and is thought to be due to particle formation between the cryoglobulin and fibrinogen. Markedly elevated leukocyte counts not confirmed by standard methods should alert the laboratory that a cryoglobulin may be present.
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Cryoglobulinemia is an uncommon cause of renal disease and often occurs in patients with hepatitis C virus (HCV) infection. We report a case of membranoproliferative glomerulonephritis in a patient with cryoglobulinemia, which was not associated with HCV infection or any identifiable etiology.
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A 45-year-old Japanese man with Sjöogren's syndrome developed recurrent skin ulcers, palpable purpura, and dyspnea. Serum mixed-type cryoglobulin level was elevated. A biopsy of his skin lesion showed the characteristic leukocytoclastic vasculitis of mixed-type cryoglobulinemia. Dyspnea, skin ulcers, and purpura resolved along with a reduction in the serum cryoglobulin level after prednisolone administration. This patient demonstrated cryoglobulinemia-associated vasculitis, as well as possible cryoglobulinemia-associated pulmonary symptoms.(Internal Medicine 36: 62-67, 1997)
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Membranoproliferative glomerulonephritis
Cryoglobulins
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Mesangial proliferative glomerulonephritis
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