Rapidly progressive glomerulonephritis (RPGN) is a type of nephritic syndrome characterized by extensive glomerular crescent formation in over 50% of glomeruli that can progress to end-stage renal disease within weeks to months if untreated. It has several etiologies including post-infectious, autoimmune, drug-induced, and underlying connective tissue diseases. Clinically it presents with hematuria, proteinuria, renal dysfunction and can involve pulmonary hemorrhage. Diagnosis involves lab tests, serology and renal biopsy showing immune complex or antibody deposition. Treatment aims to suppress inflammation and immune response with corticosteroids, immunosuppressants, and plasma exchange depending on

1. RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS
Presented by: Minel Aşcıoğlu 220i-2a MPF-2
Azerbaijan Medical University

2. OBJECTIVES:
•Definition
•Etiology
•Pathogenesis
•Clinical presentation
•Diagnostics
•Treatment

3. • Rapidly progressive
glomerulonephritis (RPGN), a
type of nephritic syndrome, is
a pathologic diagnosis
accompanied by extensive
glomerular crescent formation
(ie, >50% of sampled glomeruli
contain crescents which can be
seen in a biopsy specimen)
that, if untreated, progresses
to end-stage renal disease over
weeks to months.
DEFINITION

4. ETIOLOGY:
• In addition to primary RPGN, secondary RPGN may occur in
association with an infectious process including
poststreptococcal glomerulonephritis, infective endocarditis,
occult visceral sepsis, hepatitis B infection with vasculitis and/or
cryoglobulinemia or secondary to a systemic illness, systemic
lupus erythematosus, Henoch-Schonlein purpura, systemic
necrotizing vasculitis, malignancy, rheumatoid vasculitis;
• drugs such as penicillamine, hydralazine, allopurinol (with
vasculitis), rifampin;
• or superimposed on another primary glomerular disease like
membranoproliferative glomerulonephritis type 2, membranous
glomerulonephritis or immunoglobulin A nephropathy.

5. Comprehensive Clinical Nephrology p.194

6. • The primary feature of
crescentic glomerulonephritis
is the rupture of the basement
membrane followed by extra
capillary fibrin precipitate, and
this, followed by the
proliferation of parietal cells
and formed capsular
proliferate in a crescent shape.
Hence, the name of crescentic
GN.
Pathogenesis

7. Type-1: Antiglomerular basement membrane antibody disease
• Anti-glomerular basement membrane (GBM) antibody disease is an
autoimmune glomerulonephritis and accounts for up to 10% of RPGN
cases.
• It may arise when respiratory exposures (eg, cigarette smoke, viral upper
respiratory infection) exposes alveolar capillary collagen, triggering
formation of anticollagen antibodies. The anticollagen antibodies cross-
react with GBM, triggering a cell-mediated inflammatory response in the
kidneys and usually the lungs.
• The term Goodpasture syndrome refers to a combination of
glomerulonephritis and alveolar hemorrhage in the presence of anti-
GBM antibodies. Glomerulonephritis without alveolar hemorrhage in the
presence of anti-GBM antibodies is called anti-GBM glomerulonephritis.
Immunofluorescent staining of renal biopsy tissue demonstrates linear
IgG deposits.

8. • Immune complex RPGN complicates numerous infectious and
connective tissue disorders and also occurs with other primary
glomerulopathies such as:
• APSGN
• SLE
• Henoch-Schonlein purpura
• IgA nephropathy
• Immunofluorescent staining demonstrates nonspecific granular
immune deposits. The condition accounts for up to 40% of RPGN
cases. Pathogenesis is usually unknown.
Type-2: Immune complex RPGN

10. • Pauci-immune RPGN is distinguished by the absence of immune
complex or complement deposition on immunofluorescent
staining.
• It constitutes up to 50% of all RPGN cases.
• Almost all patients have elevated antineutrophil cytoplasmic
antibodies (ANCAs), usually ANCA(+) systemic vasculitis is the
cause.
• Wegener glanulomatosis
• Segmental glomerular necrosis is typical in this pathology.
• Treatment: with corticosteroids
Type-3: Pauci-immune RPGN

12. •Rapid loss of renal function, proteinuria,
•Hematuria,
•oligouria, anuria,
•Rapid rise of creatinine over days or weeks and azotemia
•Edema, hypertension
•Similar symptoms of glomerulonephritis
•Patients with anti-GBM antibody disease may have pulmonary
hemorrhage, which can manifest with hemoptysis
•Arthralgias, skin rash, pleurisy, pericarditis, and more rarely
cerebritis may be associated with lupus nephritis.
CLINICAL PRESENTATION

13. • Testing includes serum creatinine, urinalysis, complete blood count
(CBC), serologic tests, and renal biopsy. Diagnosis is usually by
serologic tests and renal biopsy.
• Serum creatinine is almost always elevated.
• Urinalysis shows hematuria is always present, and RBC casts are
usually present.
• On CBC, anemia is usually present, and leukocytosis is common.
• Serologic testing should include anti-GBM antibodies (anti-GBM
antibody disease); cryoglobulins (immune complex RPGN); and
antineutrophil cytoplasmic antibodies (ANCA) titers (pauci-immune
RPGN).
• Renal biopsy and immunofluorescent staining test is conducted.
DIAGNOSIS:

14. • Anticoagulants- to reduce fibrin
• Corticosteroids
• Cyclophosphamide
• Plasma Exchange: is recommended for anti-GBM antibody disease.
• Treatment varies by disease type. Therapy should be instituted early,
ideally when serum creatinine is < 5 mg/dL (442 micromol/L) and
before the biopsy shows crescentic involvement of all glomeruli.
Even patients with kidney involvement and higher creatinine levels
should be aggressively treated if they do not require
immediate renal replacement therapy.
• Dialysis and kidney transplantation also among treatment options.
TREATMENT:

15. REFERENCES:
• Crescentic Glomerulonephritis, Malvinder S. Parmar, Khalid Bashir,
StatPearls Publishing; (2022 Jan.) PMID: 28613478
• Rapidly progressive crescentic glomerulonephritis: Early treatment is
a must, Gabriella Moroni, Claudio Ponticelli; (Epub 2014 Mar 19.),
PMID: 24657897
• https://www.ncbi.nlm.nih.gov/books/NBK557430/
• https://www.msdmanuals.com/professional/genitourinary-
disorders/glomerular-disorders/rapidly-progressive-glomerulonephritis-
rpgn#:~:text=Rapidly%20progressive%20glomerulonephritis%20is%20acute,
serologic%20tests%2C%20and%20renal%20biopsy.
• Comprehensive Clinical Nephrology, John Feehally, 6th edition, 2019, p.194
• Tusem patholoji, 2017, Uz Dr. Emrullah Beyazyıldız, Üriner sistem patolojisi

16. THANK YOU!
Presented by: Minel Aşcıoğlu 220i-2a MPF-2
Azerbaijan Medical University