Nephropathology
   
Case 118
Diagnosis
 
     
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Diagnosis: Cryoglobulinemic Glomerulonephritis

Cryoglobulins are single or mixed immunoglobulins that undergo reversible precipitation at low temperatures. Cryoglobulinemia is characterized by the presence of cryoglobulins in the serum. This may result in a clinical syndrome of systemic inflammation (most commonly affecting the kidneys and skin) caused by cryoglobulin-containing immune complexes. Cryoglobulinemia may be classified based on cryoglobulin composition in: Type I cryoglobulinemia, or simple cryoglobulinemia: it is the result of a monoclonal immunoglobulin, usually immunoglobulin M; types II and III cryoglobulinemia (mixed cryoglobulinemia) contain rheumatoid factors, which are usually IgM; the actual rheumatoid factors may be monoclonal (type II cryoglobulinemia) or polyclonal (type III cryoglobulinemia) immunoglobulin. Types II and III cryoglobulinemia represent 80% of all cryoglobulins (Tritsch AM. Cryoglobulinemia. In eMedicine. Consulted on December 27th, 2015 [Link]). Membranoproliferative GN is the most common morphological picture in renal involvement for mixed cryoglobulinemia.

In our case cryoglobulins were evidenced in the serum, but hepatitis, other infections, neoplastic or autoimmune diseases were not detected, and it was considered mixed "essential" (or idiopathic). However, it is very important a long follow-up because many cases considered "essential" will show an underlying cause.

Visit the chapter: Amyloidosis... and Cryoglobulinemia of our Tutorial.

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References

  • Boumitri C, Haddad FG, Rondla C, El-Sayegh S, El-Charabaty E. Cryoglobulinemic Glomerulonephritis as a Presentation of Atypical Post-Infectious Glomerulonephritis. J Clin Med Res. 2016;8(1):47-51. [PubMed link]
  • Tritsch AM. Cryoglobulinemia. In eMedicine. Consulted on December 28th, 2015 [Link]
  • Ojemakinde K, Turbat-Herrera EA, Zeng X, Gu X, Herrera GA. The many faces of cryoglobulinemic nephropathy: a clinico-pathologic study of 47 cases with emphasis on the value of electron microscopy. Ultrastruct Pathol. 2014;38(6):367-76. [PubMed link]
  • Kowalewska J, Okoń K, Szynaka B, Naumnik B. Expression of macrophage markers in cryoglobulinemic glomerulonephritis - a possible role of CXCL9. Adv Med Sci. 2013;58(2):394-400. [PubMed link]
  • Hiramatsu R, Hoshino J, Suwabe T, Sumida K, Hasegawa E, Yamanouchi M, Hayami N, Sawa N, Takaichi K, Ohashi K, Fujii T, Ubara Y. Membranoproliferative glomerulonephritis and circulating cryoglobulins. Clin Exp Nephrol. 2014 Feb;18(1):88-94.

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