CASE PRESENTATION

Angel Sara Thangamuni

Nirmanie Hettiarachchi

Sem 10 Gr 11
A 45-year-old male patient presented with complaints of
fever and myalgias for 7 days duration, paresthesias and
weakness of both hands and feet for 4 days, cough and
breathlessness and wheeze for 4 days duration. He had
difficulty in holding objects with the hands and difficulty in
walking.
What additional information do you need ?
 MEDICAL HISTORY

● He had history of sinusitis in the past underwent sinus

surgery twice; 7 years and then 2 years ago.
● He was a known asthmatic on intermittent inhaler use
since 5 years.
● He had history of exacerbation of asthma one month
back, treated with Methylprednisolone, Monteleukast,
IV antibiotics and other supportive measures.
● He had no history of DM, HTN, CAD in the past.
What’s your
next step in
Diagnosis?
 VITALS & PE

● His general examination was unremarkable.

● PR = 108/minutes
● RR = 28/minutes
● BP = 120/70 mm Hg.
● Respiratory system examination - bilateral expiratory Ronchi.
● Nervous system examination - bilateral asymmetrical distal muscle
weakness with sensory loss.
● Deep Tendon Reflexes were not elicitable in both upper and lower limbs,
except the Biceps reflexes.
● Other system examination were normal.
 CBC

● HB = 11.7 gm/dl,
● WBC = 21,700 cells/mm3
● Platelet count = 3.0 lakhs/mm3
● ESR = 70 mm
● Absolute eosinophil count =
4000cells/mm3.
 OTHER TESTS

● Renal function tests were normal but had mild Hepatopathy.

● Creatinine phosphokinase was 532U/L (normal upto 200 U/L)
● CPK-MB is normal
● Trop T-test was negative.
● There were no ova and cysts in stools.
● Smear for microfilaria was negative.
● ANA, anti dsDNA and c-ANCA were negative.
● P-ANCA (perinuclear antineutrophil cytoplasmic antibody) (ELISA)
was positive.
 IMAGINGS

● ECG = Sinus tachycardia

● Echocardiogram = hypokinesia in septum and anterior
wall.
● Coronary angiogram = mild single vessel disease in Left
anterior descending coronary artery territory.
● Chest radiograph was normal.
● Nerve conduction Studies were suggestive of mononeuritis
multiplex.
● There was reversible airway obstructive abnormality on
spirometry.
● Bone marrow study revealed increased number of eosinophils
and their precursors.
● There was no blast prominence.
● Nerve biopsy showed features of chronic axonopathy with
patchy demyelination
● Muscle biopsy showed infiltration by eosinophilic infiltrate
with features of eosinophilic vaculitis.
 2) Nerve biopsy shows patchy
demyelination of Neurons

1) Bone marrow aspirate showing

marrow eosinophilia
3) Muscle biopsy shows
infiltration by eosinophils
WHAT’S YOUR DIFFERENTIAL
DIAGNOSIS?
● Goodpasture Syndrome
● Granulomatosis with Polyangiitis (Wegener Granulomatosis)
● Leukocytoclastic Vasculitis
● Microscopic Polyangiitis
● Polyarteritis Nodosa
● Churg-Strauss Syndrome
● Asthma
● Hypereosinophilic syndrome
● Microscopic polyangiitis
● Polyarteritis nodosa
● Drug reactions
● Bronchocentric granulomatosis
● Fungal and parasitic infections
● Malignancy.
WHAT’S YOUR FINAL
DIAGNOSIS?
CHURG-STRAUSS
SYNDROME
 TREATMENT

● Patient was treated with IV Methyl Prednisolone 1gm once a day for 3 days
and IV Cyclophosphamide 750 mg (15 mg/kg) once in 2 weeks x 3 doses, once
in three weeks x 3 doses.
● Followed by maintenance with Azathioprine 100mg once daily and oral
Prednisolone in tapering doses.
● And other medication with aspirin, statins, ACE inhibitors, isosorbide
mononitrate, physiotherapy.
● Patient improved and able to walk without any support from a bed bound
state.
● No recurrence of symptoms since two years of follow up.
 Eosinophilic Granulomatosis with Polyangiitis (EGPA) |
Churg-Strauss Syndrome

Rare systemic necrotizing vasculitis

● affects small-to-medium-sized vessels
● associated with severe asthma
● blood and tissue eosinophilia
Etiology

● Hereditary
● Drug induced -
● Leukotrienes receptor antagonist (-lukast)
● Cocaine
● Covid 19 vaccination
●
 EGPA: Stages of disease

● Atopic disease
● Allergic rhinitis
● Asthma
 Signs and symptoms

Classical triad

1. Asthma and/or allergic rhinitis

2. Eosinophilic lung disease similar to pneumonia
3. Systemic vasculitis

● Mononeuritis multiplex
● Peripheral neuropathy
● Peripheral eosinophilia
 Other clinical features
most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal,
and peripheral nerve involvement

Lung involvement
● Most common organ involved
● Asthma
● Pulmonary opacities on imaging
● Pleural effusion (with eosinophils)
● Hemoptysis secondary to pulmonary alveolar
hemorrhage (alveolar capillaritis)

Ears nose and throat (ENT)

● Otitis media allergic rhinitis
● Recurrent sinusitis
● Nasal polyps

Peripheral neuropathy
● Mononeuritis multiplex (most frequent form,
occurring in as many as 77% of patients)
Cardiac involvement
● Significant cause of mortality
● heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial
infarction

Skin
● Leukocytoclastic angiitis with palpable purpura
● Livedo reticularis, skin necrosis and gangrene, digital ischemia, urticaria, and
subcutaneous nodules

Renal :
● Hypertension
● Signs of uremia and advanced kidney failure

Gastrointestinal
● GI bleeding ● Gastroenteritis
● Bowel ischemia and perforation ● Appendicitis
● Pancreatitis
Constitutional symptoms
● Especially occurring in the Vasculitic phase
● Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias
(52%)
 Laboratory Studies
CBC -eosinophilia, anemia.

The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) - usually elevated.

In patients with renal involvement,

blood urea nitrogen (BUN) and serum creatinine levels are elevated.
Urinalysis - abnormal urine sediment, proteinuria, microscopic hematuria, and red blood cell casts.

Antineutrophil cytoplasmic antibodies (ANCAs) are present in approximately 40% of patients with EGPA. Most of these patients are
perinuclear-ANCA (p-ANCA)–positive (antimyeloperoxidase antibodies). [29]

Other immunologic test results are as follows:

● Elevated serum IgE levels
● Hypergammaglobulinemia
● Positive results for rheumatoid factor at low titer
● Elevated levels of eosinophil cationic protein (ECP),
On bronchoalveolar lavage (BAL), eosinophilia is evident in 33% of cases.
 Imaging Studies

● Chest radiography and chest computed tomography (CT) ,

● Electrocardiogram (ECG) for cardiac manifestations
● Gastrointestinal endoscopy for GI bleeding
● Electromyelography (EMG) and nerve conduction studies for peripheral
neuropathies

Transient pulmonary infiltrate consolidation or ground glass opacities

 Histologic Findings

Granulomas are composed of a central eosinophilic core surrounded radially by

macrophages and epithelioid giant cells.found especially in the lung

● Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).

 Biopsy

If local organ involvement exists, obtaining a biopsy of that organ is most helpful in
confirming the diagnosis.

● Skin
● Lung - Open or video-assisted thoracoscopic biopsy is
preferred over transbronchial
● Renal
● Nerve
● Muscle - Muscle biopsy has a sensitivity of about 67% in
detecting systemic vasculitis

If no localizing finding exists, obtaining nerve or muscle biopsy may

be considered
Diagnostic criteria/classification
Prognosis- Five factor score
 Treatment

● Rituximab ( in treatment of steroid-resistant cases)

Maintenance therapy - ● anti-IgE monoclonal antibody omalizumab
● Mepolizumab
Azathioprine, methotrexate, ● Benralizumab,
References

https://emedicine.medscape.com/article/333492-differential

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4129332/

https://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=745&Disease_Disease_S
earch_diseaseGroup=churg-strauss&Disease_Disease_Search_diseaseType=Pat&Disease(s)%20concer
ned=Churg-Strauss-syndrome&title=Churg-Strauss-syndrome&search=Disease_Search_Simple#:~:text
=Differential%20diagnosis,and%20parasitic%20infections%2C%20and%20malignancy
.

Vaglio, A., Buzio, C. & Zwerina, J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss):
state of the art. Allergy 68, 261–273 (2013).

Jennette, J. C. et al. 2012 Revised International Chapel Hill consensus conference nomenclature of
vasculitides. Arthritis Rheum. 65, 1–11 (2013).
Thank you