A rare case of granulomatosis with polyangitis induced non bacterial thrombotic endocarditis
mimicking as infective endocarditis : A CASE REPORT
INTRODUCTION
Granulamatosis with polyangitis (GPA)is an ANCA associated vasculitis that causes inflammation
of small to medium sized blood vessels primarily in upper airways ,lower airways and
kidneys .Hallmark features are necrotizing granulomas and pauci-immune vasculitis. Cardiac
involvement is rare, with pericarditis being the most frequent complications but here patient
presented with non bacterial thrombotic endocarditis (NBTE) and presentation mimics as Infective
endocarditis (IE).
CASE REPORT:
A 42-year-old male was admitted to our hospital with complaints of dry cough, low grade fever,
joint pain(b/l small and large joints) not associated with morning stiffness, generalized
bodyache ,weight loss since 1 month and decreased hearing since 10 days.Patient was
stable,febrile with B.P.- 140/90mmhg and cxr suggestive of left sided hilar opacity(?)
and investigations are as follows:
Pretreatment
Post treatment
HB/WBC
11.3/11.6
9
N/L/E/B
74/13/6(7
36)/0
(701)
PLT
600000
584000
U/CREAT
154/4.44
177/5.95
175/6
115/3.19
Na/K/Ca/
PO4
136/6
137/6.1
137/5.7
141/5/8.6/
4.6
TSB/
SGPT
0.36/51
0.34/36
0.33/25
ESR/CRP
114/4.8
RA/ASO
8 (+)/0
PT/APTT/
INR/DDIMER
12.8/24.3/
0.99/
3116
URINE R/
M
Pus cells6/hpf
Blood –
present
Rbc10-15/hpf
Dysmorph
ic
Urine
protein-30
mg/dl
T.P/A/G
8.1/3.5/4.
6
12.2/5.77
(596)
71/23/3
286000
1.3
Pus cells :
1 cells/hpf
Isomorphi
c rbcs -5/
hpf
Dysmorph
ic rbcs
-<1
Protein:
100 mg/dl
p/c ->0.5
a/c-.>300
7.5/3.5/4
S.IGE – 636 IU/L
ON ENT EXAM.: S/O SEROUS OTITTIS MEDIA
Pretreatment
2D - ECHO
1.TTE) –MV : FLAIL AMVL ECHOGENIC
STRUCTURE ATTACHED TO PML TIP (?)
VEGETATION (?) CHORDAL TISSUE ( SIZE:6x3
mm) MILD MR ( ECCENTRIC ANTERIOR
DIRECTED JET) TRIVIAL AR MILD TR RVSP20 LVEF – 60%
2.TEE – MV-SMALL ECHOGENIC MASS AT
TIP OF 2x3 AMVL AND 4x5 PMVL MODERATE
MR (25- 30% ), ANT JET OF MR
HRCT THORAX -(20/1/23)
MULTIPLE ROUNDED NODULES/SOFT
TISSUE LESIONS ARE SEEN IN RIGHT
UPPER( APICAL 23 x 23 ),MIDDLE LOBE AND
BILATERAL LOWER LOBES WITH LEFT
LOWER LOBE SUPERIOR SEGMENT (25 x 26
MM) (MOSTLY PERIPHERAL AND
SUBPLEURAL REGION
USG KUB –
B/L RAISED CORTICAL ECHOGENICITY
PRESENT WITH CMD PRESERVED
3 BLOOD CULTURES WERE NEGATIVE AND DIAGNOSED AS INFECTIVE ENOCARDITIS WITH
ACUTE GLOMERULONEPHRITIS WITH SEPTIC EMBOLI(?).AS RFTS SUGGESTIVE OF RAPIDLY
PROGRESSIVE GLOMERULONEPHRITIS (RPGN) SO FURTHER EVALUATION WAS DONE WITH
24 HR URINE PROTEINS: 1.2g (NEPHRTIC RANGE)
S.Ca/PO4 -8.8 / 3.7
s.C3-191 mg/dl S.C4- 53.6mg/dl
ANA : 0.312 , ds-DNA : 20.878
C-ANCA - > 100 U/ml (0.01 – 5 U/ml)
p-ANCA – 1.05 (U/ml) (0.01 – 5 U/ml)
ON RENAL BIOPSY : IMMUNE COMPLEX MEDIATED CRESCENTIC GLOMERULONEPHRITIS (C
ANCA ASSOCIATED) WITH SEGMENTAL GLOMERULONECROSIS (ON IF –NEGATIVE FOR C3
AN C1q anti sera)
FINAL DIAGNOSIS :C-ANCA VASCULITIS GPA WITH NBTE WITH RPGN
TREATMENT :
INDUCTION THERAPY
• ( INJ. MPS 500 mg/day for 3 days)+ 2
ROUNDS HEMODIALYSIS 48 HRS APART
• INJ.CYCLOPHOSPHAMIDE 800
MG(Wt-64kg) + INJ.MESNA 200 mg X 7
CYCLES.
• STEROIDS ( INJ. MPS 40MG / DAY)
presently on
MAINTAINANCE THERAPY:
T. PRED. (5MG ) OD + TAB. AZATHIOPRINE
100 MG HS AND PATIENT IS ON FOLLOWUP.
POST TREATMENT
HRCT THORAX( 9/5/23)
RIGHT SIDED MILD LOCULATED PLEURAL
EFFUSION ,FOCAL FIBROTIC OPACITY IN
POSTERIOR SEGMENT OF RIGHT UPPER
LOBE
2D ECHO TTE (14/3/23)
MVP WITH MODERATE MR WITH HEALED
VVEGETATIONS ON PMVL TIP.
Conclusion and discussion:
Patient is diagnosed as c-anca associated vasculitis with upper respiratory
tract(ottitis media).,Lower respiratory tract (pulmonary nodules) and kidney (RPG N)
suggestive of GPA. The ACR/EULAR 2022 final criteria :
bloody nasal discharge, nasal crusting or sino- 3
nasal congestion
cartilaginous involvement
2
conductive or sensorineural hearing loss
1
cytoplasmic antineutrophil cytoplasmic
antibody (ANCA) or anti-proteinase 3 ANCA
positivity
5
pulmonary nodules, mass or cavitation on
chest imaging
2
granuloma or giant cells on biopsy
2
inflammation or consolidation of the nasal/
paranasal sinuses on imaging
1
pauci-immune glomerulonephritis
1
P anca
-1
eosinophil count ≥1×109 /L
-4
.After excluding mimics of vasculitis, a patient with a diagnosis of small- or
medium-vessel vasculitis could be classified as having GPA if the cumulative score
was ≥5 points ,here there are 9 points. ECHO do not differ between positive and
negative ANCA IE, but there seems to be more renal involvement with a positive
ANCA and can present as pulmorenal syndrome but GPA can also present as
NBTE . Although in pauci-immune glomerulonephritis by definition, there is a
paucity of immune deposits on immunofluorescence microscopy and electron
microscopy, a significant proportion of cases may indeed show Ig deposits on
histopathological examination .It is important to differentiate IE and GPA ,as
treatments are contradictory for both of these and both carries high mortality if left
undiagnosed or misdiagnosed especially in severe GPA.
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