Dmitrieva M.V.1, Letkovskaya T.A.2, Chyzh K.A.2, Bragina Z.N.2
Pauci-immune glomerulonephritis with crescents: clinical, morphological and prognostic analysis
1City Clinical Pathologoanatomic Bureau, Minsk, Republic of Belarus
2Belarusian State Medical University, Minsk, Republic of Belarus
Vestnik VGMU. 2018;17(6):64-76.
Pauci-immune glomerulonephritis (PIGN) belongs to the most severe forms of the disease with a steady decrease of kidney function, variable clinical picture, which greatly complicates the diagnosing. The prognosis of the disease often depends on the timely made diagnosis.
Objectives. To give a clinical, morphological and prognostic characteristics of РIGN with the crescents in the glomeruli.
Material and methods. 33 cases of PIGN with crescents in the glomeruli were used in this study. A retrospective analysis of the kidney biopsy results as w-ell as of clinical and laboratory data and the evaluation of long-term results concerning kidney function and lethal outcome were made. The histological slides were stained with hematoxylin-eosin PAS, Masson trichrome, Congo-red, Jones silver. A direct immunofluorescence study was conducted with G,A,M immunoglobulins, C3c and C1q complement components, fibrinogen, κ and λ light chain components (Polyclonal Rabbit Anti-Human / FITC, Dako, Denmark).
Results. Gender-age analysis showed that half of the cases were 50 and more years old. There were 2,3 times more females than males. The clinical manifestations of PIGN did not depend on the age categories and the degree of extracapillary damage (diffuse or focal). Isolated GN pattern was characterized by more pronounced proteinuria (p=0,0013) compared with GN associated with systemic vasculitis. An important characteristic in determining the disease prognosis was the crescent score (CS). In those cases when the crescent score was more than 1,5, the risk of death increased 2,3 times (95% CI 1.16-4.58), and the risk of progression up to the end stage of chronic renal failure was 4.6 times higher (95% CI - 2,46-8,59). The kidney function survival analysis revealed its one-year survival in 67%, 3- and 5-year survival in 38 and 14% of patients, respectively. The lethal outcome was associated with systemic manifestations of vasculitis, 80% of which occurred within the first four months of follow-up.
Conclusions. The diagnosis of PIGN requires an increased attention to patients regardless of clinical and morphological manifestations. The evaluation of extracapillary proliferation (EP) degree in renal glomeruli with calculating CS is important for the prognostication with regard to kidney function.
Key words: pauci-immune glomerulonephritis, extracapillary proliferation, kidney biopsy, clinical and morphological manifestations, prognosis.
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Information about authors:
Dmitrieva M.V. – pathologist of the pathoanatomical department of general pathology No. 1, City Clinical Pathologoanatomic Bureau;
Letkovskaya T.A. – Candidate of Medical Sciences, associate professor, head of the Chair of Pathologic Anatomy, Belarusian State Medical University;
Chyzh K.A. – Candidate of Medical Sciences, associate professor of the Chair of Internal Diseases No. 2, Belarusian State Medical University;
Bragina Z.N. – Candidate of Medical Sciences, associate professor of the Chair of Pathologic Anatomy, Belarusian State Medical University.