CONCURRENT ACUTE

POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS AND
ACUTE RHEUMATIC CARDITIS IN A
PATIENT
dr. Alfredo
Mentors:
dr. R. Rochmanadji W, SpA(K), MARS
Dr. dr. M. Heru Muryawan, SpA(K)
Dr. dr. Omega Mellyana, SpA(K)
ACUTE RHEUMATIC FEVER
 Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a
sequela of streptococcal infection.
 ARF has extremely variable manifestations, and remains a clinical syndrome for which no
specific diagnostic test exists.
 Persons who have experienced an episode of ARF are predisposed to recurrence following
subsequent group A streptococcal infections.
 The most significant complication of ARF is rheumatic heart disease, which usually occurs
after repeated bouts of acute illness.
PREVALENCES
 Pharyngitis

Impetigo

Supurative Cellulitis

Myositis

Streptococcus Beta Hemolyticus Group A

Pneumonia

Rheumatic Fever

Non Supurative

Glomerulonephritis
PATHOGENESIS
CLINICAL MANIFESTATION
JONES CAFÉ PAL CRITERIA
Low-risk population is defined as an
acute rheumatic fever incidence of <2 per
100,000 school-aged children or all
age rheumatic heart disease prevalence
of ≤1 per 1000 population per year.
Those not included in the low-risk
population are defined as moderate or
high risk depending upon their reference
population.
GOALS OF TREATMENT
 Eradication of group A beta-hemolytic Streptococcus (GAS)
 Symptomatic relief of acute disease manifestations (eg, arthritis, fever)
 Manage rheumatic heart disease (RHD; eg, carditis, heart failure) if present
 Manage chorea if present
 Prophylaxis against future GAS infection to prevent progression of cardiac
disease
 Provision of education for the patient and patient's caregivers
 ANTIBIOTIC

National Heart Foundation of New Zealand. New Zealand Guidelines for Rheumatic Fever: Diagnosis, Management and Secondary
Prevention of Acute Rheumatic Fever and Rheumatic Heart Disease: 2014 Update. 2014
SYMPTOMATIC RELIEF
 Paracetamol of 60 mg/kg/day divided into 4-6 doses (max 4g)
 Codein 0,5-1 mg/kg per 4-6 hr
 NSAID administration could mask symptoms of ARF, so it should be avoided until proper
diagnostic measure is achieved
 In case of NSAID usage, Naproxen, Aspirin or Ibuprofen could be given
RHEUMATIC HEART DISEASE
MANAGEMENT
 Supportive, bed rest, escalating mobility
 Depends on the severity
 Diuretic -> reduce preload
 ACEi -> reduce afterload
RHEUMATIC HEART DISEASE
MANAGEMENT
RHEUMATIC HEART DISEASE
MANAGEMENT
MANAGEMENT OF CHOREA
 Usually self-limiting and doesn’t need specific therapy
 Therapies of Carbamazepin 7-20 mg/kg/day 3dd, Valproic Acid 15-20 mg/kg 3dd could be
considered if symptoms limit daily activities
 Avoid Haloperidol or other neuroleptic drugs -> extrapyramidal side effects
 SECONDARY PREVENTION
 To prevent recurrency of ARF
 To prevent progression to rheumatic heart disease
 Recommended for patients with ARF with and or without Carditis manifestation
 Given for 10 years after last episode or until age 40 years for patients with carditis with
permanent cardiac abnormality.
 Also, given for 10 years after last episode or until age 21 years for patients with carditis but
with no permanent cardiac abnormality
 Also, given for 5 years after last episode or until age 21 years for patients without carditis.

Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association
Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology,
and the Interdisciplinary Council on Quality of Care and Outcomes Research. Circulation. 2009; 119(11):1541-1451
 SECONDARY PREVENTION

Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: a scientific statement from the American Heart Association
Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology,
and the Interdisciplinary Council on Quality of Care and Outcomes Research. Circulation. 2009; 119(11):1541-1451
ACUTE POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS
DEFINITION
 Post-streptococcal glomerulonephritis (PSGN) is an immunologically-mediated sequela of
pharyngitis or skin infections caused by nephritogenic strains of Streptococcus pyogenes. S.
pyogenes are also called group A Streptococcus (group A strep).
CLINICAL FEATURES
 Edema (often pronounced facial and orbital edema, especially on arising in the morning)
 Hypertension
 Proteinuria
 Macroscopic hematuria, with urine appearing dark, reddish-brown
 Complaints of lethargy, generalized weakness, or anorexia
LAB FINDINGS
 Mild normocytic normochromic anemia
 Slight hypoproteinemia
 Elevated blood urea nitrogen and creatinine
 Elevated erythrocyte sedimentation rate
 Low total hemolytic complement and C3 complement
 Patients usually have decreased urine output. Urine examination often reveals protein (usually
<3 grams per day) and hemoglobin with red blood cell casts.
TREATMENT
 focuses on managing hypertension and edema.
 Additionally, patients should receive penicillin (preferably penicillin G benzathine) to
eradicate the nephritogenic strain. This will prevent spread of the strain to other people.
 RARE CASE OF CONCURRENT
ACUTE POSTSTREPTOCOCCAL
GLOMERULONEPHRITIS AND
ACUTE RHEUMATIC CARDITIS IN
A PATIENT
: Abdulkader HB, Unni NV, Kumar KV, Sreekumar P. Concurrent acute poststreptococcal glomerulonephritis and
acute rheumatic carditis in a patient. Asian J Pediatr Nephrol 2021;4:85-8.
INTRODUCTION
 Acute poststreptococcal glomerulonephritis (APSGN) and acute rheumatic fever (ARF) are
both well‑recognized nonsuppurative sequelae of group A beta-hemolytic streptococcus
infections.
 The serotypes of group A streptococci can be divided into those with rheumatogenic and
nephritogenic potential.
 Poststreptococcal glomerulonephritis (GN) and rheumatic fever (RF) have different
epidemiology and immunological features; their concurrent development in the same patient is
very rare,[3] because of different pathophysiologic mechanisms.[
CASE PRESENTATION
 Gender : Male
 Age : 15th
 history of fever 3 weeks ago associated with sore throat.
 Twenty days after the onset of fever, he noticed puffiness of the face followed by progressive
worsening of edema involving the rest of the body. He had bilateral pedal edema and
abdominal distension, associated with reduced urine output. He had arthralgia involving both
knee joints and noticed cola-colored urine. He had gradual worsening of dyspnea and
tachypnea. There was a weight gain of 8 kg.
PHYSICAL EXAMINATION
 Febrile
 BP : 106/68 mm Hg (corresponding to 90th centile)
 Height : 162 cm
 Weight : 42 kg
 BMI : 15.4
 BSA : 1.43
 Palor, facial and pedal edema
 grade 3/6 pansystolic murmur in the mitral area radiating to the axilla. No other murmurs
could be appreciated. no clinical signs of infective endocarditis or congestive cardiac failure.
 There was no evidence of subcutaneous nodules, erythema marginatum, or features of arthritis
LABORATORY FINDINGS
 Serum creatinine :1.8 mg/dL.
 hemoglobin :9.9 g/dL
 white blood cell count: 8100/mm3
 platelet count : 242,000/mm3
 erythrocyte sedimentation rate (ESR): 44 mm/h
 C‑reactive protein : 103.5 mg/dL
 Serum albumin : 2.8 g/dL.
 Urinalysis showed microscopic hematuria and proteinuria (6.1 g/day).
 He had elevated antistreptolysin O (ASO) titers (>800 IU/mL) and hypocomplementemia
(serum C3 45 mg/dL and C4 20 mg/ dL).
X-RAY CHEST
 bilateral blunting of costophrenic angles
ECG
 prolonged PR interval (0.32 s)
ULTRASONOGRAM OF THE
ABDOMEN
 bulky kidneys (right kidney of 11.6 cm and left kidney of 11 cm) with diffusely increased
echotexture without evidence of hydroureteronephrosis.
CULTURES
 Blood and urine cultures were sterile.
 The throat swab showed no growth.
 BIOPSY
 Renal biopsy was done after initiation of dialysis and showed features of diffuse exudative
proliferative GN with neutrophilic exudate [Figure 1a‑c].
 Immunofluorescence revealed granular capillary wall staining for immunoglobulin G (3+) and
C3 (2+). The picture was consistent with infection-related GN

Kidney biopsy showing (a) enlarged glomeruli, interstitial edema, interstitial inflammation, and red blood cell casts in the
tubular lumina (H and E, ×20); (b) diffuse endocapillary proliferation (yellow arrow, (H and E, ×40); and (c) mononuclear
and neutrophilic exudate (Green arrow, H and E, ×40)
ECHOCARDIOGRAPHY
ECHOCARDIOGRAPHY
 dilated left atrium and left ventricle,
 right coronary cusp prolapse,
 severe aortic regurgitation (AR).
 aortic valve showed multiple jets suggestive of perforation, the anterior mitral leaflet was
thickened, and there was severe eccentric mitral regurgitation with chordal rupture
 LVEF 65%
 No evidence of vegetation
TREATMENT
 prednisolone 60 mg/day (1.5 mg/kg/day)
 oral furosemide and enalapril.
 Injection benzathine penicillin 12 lakhs IU intramuscularly was advised every 3 weeks
FOLLOW UP
 Renal functions improved gradually after initiation of steroids
 The AR improved from severe AR to mild AR over 10 days but moderate mitral regurgitation
persisted.
 At the time of discharge, his serum creatinine was 2.9 mg/dL.
 Steroids were tapered and stopped after 3 months.
 On follow‑up at the end of 6 months, serum creatinine was 0.7 mg/ dL (estimated glomerular
filtration rate of 96 ml/min/1.73 m2 based on modified Schwartz equation), ESR 15 mm at the
end of 1 h, and urine analysis showed 5 RBCs/hpf without albuminuria.
DISCUSSION
 Nephritogenic strains associated with the development of APSGN include M types 1, 4, 12, 15
(cause throat infection and APSGN), and 2, 42, 49, 55, 57, and 60 (cause pyoderma and
APSGN).
 A streptococcal strain capable of causing a well-documented pharyngitis almost always is
potentially capable of causing RF.
 M types associated with RF are 1, 3, 5, 6, 11, 12, 14, 17, 18, 19, 24, 27, 29, 30, 32, and 41.
 The lack of specific rheumatogenic strains can also explain the relatively high risk for
recurrent disease with new streptococcal infections, in contrast to poststreptococcal GN, in
which only a few nephritogenic strains appear to be capable of inducing the disease, and
hence, recurrent disease is uncommon.
DISCUSSION
 The coexistence of APSGN and ARF rarely occurs, as has been previously described.
 Simultaneous occurrence of APSGN and acute RF was first reported in 1981. After that, 17
cases have been reported. Most of the cases had acute RF preceding or after the event of
APSGN. Only six of 17 patients had simultaneous occurrence of acute RF and APSGN.
 The explanation for the co-occurrence might be that, certain strains have both nephritogenic
and rheumatogenic potential (M type 1, 12), which can result in the occurrence of both
conditions.
 Although some serotypes are both rheumatogenic and nephritogenic, it cannot be said with
certainty whether a single pharyngeal infection resulted in both APSGN and acute RF in this
case; however, the possibility of a single infection causing both PSGN and RF cannot be ruled
out.