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

SMALL VESSEL

Giant cell arteritis Takayasu arteritis.

Immune complex

MEDIUM VESSEL

lupus/rheumatoid vasculitis, HSP, Cryoglobulinemic vasculitis. Wegeners , Microscopic polyangitis Churg strauss syndrome.

PAN, Kawasakis disease.

Pauci immune

ACR Criteria for Churg-Strauss syndrome

1. 2. 3. 4. 5.

6.

Bronchial asthma Eosinophilia >10% differential count Mononeuropathy / polyneuropathy attributable to vasculitis Transient pulmonary infiltrates attributable to vasculitis Paranasal sinus abnormality 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