an interesting case of fever
TRANSCRIPT
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An interesting case of fever
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17 yr old male was admitted with c/o Fever Abdomen pain Vomiting Breathlessness for the past two weeks.
Fever – intermittent, relieved by antipyretics.
Abdomen pain – diffuse, more in the right hypochondrium
No c/o jaundice/ loose stools
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k/c/o bronchial asthma since childhood,
Breathlessness aggravated for the past two weeks
Patient was operated at 1 yr of age (?PDA)
Personal history & family history: not significant
No h/o drug intake
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Examination
Conscious, oriented, febrile, hydration fair Not pale Not icteric No cyanosis No clubbing No pedal edema Significant generalized lymphadenopathy
present (cervical, axillary, inguinal)
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Pulse: 116/min, regular BP: 110/70 mm hg CVS: S1S2+, no murmurs RS: NVBS heard, B/L wheeze heard P/A :
Soft, hepatomegaly + Liver span 16cm
CNS: normal
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Investigations
Hb: 11.9 gm% TC: 15400 cells/ cumm DC: P68L20E12 ESR: 25 mm at 1 hr Platelets: 3 lacs/ cumm Peripheral smear study:
mild leucocytosis, eosinophilia. no immature cells / parasites.
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RBS: 80mg/dl Urea: 20 mg/dl Creatinine: 0.6 mg/dl CXR: normal ECG: WNL Urine routine: normal USG abdomen: hepatomegaly Stool ova, cyst – negative.
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CRP: positive LFT:
Bilirubin: 0.6 OT: 24 PT: 22 ALP: 56 Protein: 5.6 Alb: 3.7
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QBC for MP : -ve Widal : -ve MSAT: -ve Blood C & S: no growth. Sputum c/s – no growth. Urine c/s – no growth. HIV, HBsAg, Anti HCV: negative
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Differential Diagnoses???
INFECTIONS – TB, toxoplasmosis, HIV, infectious mononucleosis.
LYMPHOID MALIGNANCY – leukemia, lymphoma.
CONNECTIVE TISSUE DISORDERS.
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Sputum for AFB: negative
CERVICAL NODE BIOPSY: patchy necrosis & infarction with focal palisading
histiocytic aggregates, moderate eosinophilic infiltrate & evidence of vasculitis.
Inflammation of walls of small blood vessels with neutrophil and lymphocyte infiltrate, fibrinoid necrosis and nuclear debris surrounding some vessels.
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ANA: negative Anti dsDNA: negative ANCA: negative RF and anti CCP: negative Serum ferritin: normal C3 C4 level : normal Ig E : >2500.
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CHEST XRAY
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CT CHEST
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HRCT CHEST
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CT CHEST : Volume reduction in left lung with
compensatory hypertrophy of the right lung.
Ground glass opacities with parenchymal infiltrates.
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CT PNS
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CT PNS – B/L maxillary sinusitis L>R.
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CECT Abdomen
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DIAGNOSIS?
Young male with fever 4 weeks and generalised lymphadenopathy.
H/O bronchial asthma. Eosinophilia. Sinusitis. Lymph node biopsy – necrotising vasculitis of small
vessels with eosinophil infiltrate. CT CHEST – pulmonary infiltrates resolved with
treatment.
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VASCULITIS - CLASSIFICATION
LARGE VESSEL Giant cell arteritis Takayasu arteritis.
MEDIUM VESSEL PAN, Kawasakis disease.
SMALL VESSEL Immune complex
lupus/rheumatoid vasculitis,
HSP, Cryoglobulinemic
vasculitis. Pauci immune
Wegeners , Microscopic polyangitis Churg strauss syndrome.
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ACR Criteria for Churg-Strauss syndrome
1. Bronchial asthma2. Eosinophilia >10% differential count3. Mononeuropathy / polyneuropathy attributable to vasculitis4. Transient pulmonary infiltrates attributable to vasculitis5. Paranasal sinus abnormality6. Extravascular eosinophils
If 4 out of 6 criteria are present patient can be classified as Churg Strauss syndrome.
Sensitivity – 85%, specificity – 99%
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TREATMENT
Pt started on oral prednisolone 1 mg/kg.
He improved symptomatically with resolution of fever ,constitutional symptoms and lymphadenopathy.
Steroid dose tapered to 0.5 mg/kg.pt discharged and adviced to review after 2 weeks.
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DISCUSSION
Described by Churg and Strauss in 1951 when they reviewed autopsy cases previously diagnosed as PAN with atypical features like asthma and extravascular granulomas.
ACR criteria published in 1990 most commonly used.
DD – wegeners granulomatosis, drug reactions, bronchocentric granulomatosis, eosinophilic granuloma, fungal,parasitic infections, malignancy.
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Incidence : 1-3 / million.
Non specific features – fever, malaise, anorexia, weight loss.
Pulmonary manifestations most common –severe asthma and pulmonary infiltrates.
Mononeuritis multiplex, allergic rhinitis and sinusitis are other common features.
Skin – purpura and cutaneous nodules.
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Nervous system – mononeuropathy, polyneuropathy, cerebral hemorrhage, infarcts.
CVS – pericarditis, myocardial infarction, cardiomyopathy
GI – eosinophilic gastroenteritis, colitis, bowel ischemia, pancreatitis, cholecystitis, GI bleed.
Kidneys – glomerulonephritis, interstitial nephritis.
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LAB INVESTIGATIONS
Eosinophilia. Elevated ESR. Elevated IgE. p ANCA positive in 48% . Biopsy –
Necrotising vasculitis of small and medium size arteries, capillaries, veins and venules.
Extravascular eosinophil infiltrates and granuloma formation.
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Glucocorticoids alone are effective in many patients.
In patients with fulminant disease unresponsive to steroids alone combination of cyclophosphamide and steroids are used.
With treatment prognosis is favourable. Myocardial involvement is the most common
cause of death.
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PULMONARY INFILTRATES WITH EOSINOPHILIA
Allergic bronchopulmonary mycoses.
Drug reactions.
Parasitic infestations.
Eosinophilia myalgia syndrome.
Loefflers syndrome. Acute eosinophilic
pneumonia. Chronic eosinophilic
pneumonia. Churg strauss
syndrome. Hypereosinophilic
syndrome
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