An interesting case of fever

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

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

Examination

Conscious, oriented, febrile, hydration fair Not pale Not icteric No cyanosis No clubbing No pedal edema Significant generalized lymphadenopathy

present (cervical, axillary, inguinal)

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

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.

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.

CRP: positive LFT:

Bilirubin: 0.6 OT: 24 PT: 22 ALP: 56 Protein: 5.6 Alb: 3.7

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

Differential Diagnoses???

INFECTIONS – TB, toxoplasmosis, HIV, infectious mononucleosis.

LYMPHOID MALIGNANCY – leukemia, lymphoma.

CONNECTIVE TISSUE DISORDERS.

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.

ANA: negative Anti dsDNA: negative ANCA: negative RF and anti CCP: negative Serum ferritin: normal C3 C4 level : normal Ig E : >2500.

CHEST XRAY

CT CHEST

HRCT CHEST

CT CHEST : Volume reduction in left lung with

compensatory hypertrophy of the right lung.

Ground glass opacities with parenchymal infiltrates.

CT PNS

CT PNS – B/L maxillary sinusitis L>R.

CECT Abdomen

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.

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.

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%

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.

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.

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.

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.

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.

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.

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

THANK YOU!!!