Introduction

Churg-Strauss described allergic angitis andgranulomatosis in 1951.1,2 It is a rare syndrome with reported annual incidence of 2.5 per millionper year in adults. It is a granulomatous vasculitisof multiple organ systems, particularly of the lung. It involves blood vessels of various types and sizes(including veins and venules), intra- and extravasculargranuloma with eosinophilic tissue infiltration. The cause of allergic angiitis and granulomatosis is not known. No data have been reported regarding the role of immune-mediated mechanism in the disease. Disease has strong association with severe asthma and peripheral eosinophilia. It is because of rarity of this disease, present case is reported.

Case report

A 45-year-old woman presented with a four dayshistory of fever, swelling over all four limbs, joint pain,myalgia and rash over legs. There was no history ofcough, breathlessness, chest pain, abdominal pain,vomiting, bleeding from any site, urinary symptoms orunconsciousness.

On examination, she was febrile with a heart rate of90/minute, blood pressure of 140/90 mmHg. She waspale, had cervical lymphadenopathy, tender pittingedema overall four limbs and papular skin lesionsover legs. Her systemic examination revealed bilaterallung fields having normal vesicular breath sounds,no cardiomegaly, abdomen soft with no ascites or organomegaly. Central nervous system examinationwas unremarkable.Based on the presentation and clinical findings, aprovisional diagnosis of viral exanthema in evolution, sepsis and vasculitis were kept.

Her complete blood count and peripheral smearrevealed total leukocyte count of 30,100/mm3 with 85%neutrophils, and hemoglobin of 7.8 g/dl. Her electrolytes,random blood sugar, urea, creatinine, CPK, chest X-ray,ECG, abdominal ultrasonography were normal.The patient received systemic prednisolone and anantimicrobial empirically along with supportivetreatment. On third day the patient developed acuteonset breathlessness. The patient did not respond tomedical treatment and died.

Autopsy was performed and histopathology reportrevealed Churg-Strauss syndrome involving intraandextravascular granuloma with eosinophilictissue infiltration of small, and medium-sized bloodvessels with predominant involvement of coronaryarteries with secondary myocardial infarction (Figs.1 and 2). Small focus of dull cortical necrosis with sorrounding viable renal tissue and small vessel wasseen in renal cortex (Figs. 3 and 4). Liver showed mixedinflammatory infiltrate of predominantly eosinophils inportal triad surrounded by normal hepatic parenchyma(Figs. 5 and 6).

Discussion

Churg-Strauss disease causes a variety of nonspecificclinical symptoms and signs. It mimics many othersystemic diseases like asthma, acute mesenteric ischemia,polyarteritis nodosa, Wegener’s granulomatosis,hypereosinophilic syndrome, leukocytoclastic vasculitis, Goodpasture syndrome and eosinophilic pneumonia.Most patients with Churg-Strauss disease have aprodromal period of many years characterized byallergic rhinitis, nasal polyposis and late onset asthmathat is often difficult to control.3 Among the early symptoms and signs, similar to that in our patient, arefever, weight loss, pain in viscera, or the musculoskeletalsystem. Striking and specific initial signs may relate toacute glomerulonephritis, polyneuritis and myocardialinfarction. The typical presentation of Churg-Strausssyndrome is a triad of skin lesions (purpura or nodules),asymmetric mononeuritis multiplex and eosinophilia ona background of resistant asthma. Some times, patientsmight also present without both eosinipilia and asthmasimilar to our patient.4 Upto 50% of patients haveabdominal symptoms due to mesenteric vasculitis.

Leukocytosis in more than 75% cases, eosinophilia,increased ESR. C-ANCA or P-ANCA is present inaround 40% of cases. Pulmonary opacities in 26-77%,no abnormalities in 25%, pleural effusion in 5-30% ofthe patients on X-ray chest.5 Pleural and pericardialeffusions due to serositis may also be seen.The American College of Rheumatology has proposed six criteria for diagnosis of Chung-Strauss syndrome.6The presence of four or more criteria yields a sensitivityof 85% and a specificity of 99.7%. These criteria are:

1. Asthma (wheezing, expiratory ronchi)
2. Eosinophilia of more than 10% in peripheral smear
3. Paranasal sinusitis
4. Pulmonary infiltrates (may be transient)
5. Histological proof of vasculitis with extravascular eosinophils

Mononeuritis multiplex or polyneuropathy.It is critically important to establish this diagnosis.A high index of suspicion remains the most importantelement in diagnosis. Diagnosis of Churg-Straussdisease is made by biopsy.7 The prognosis of untreated disease is poor; with a reported 5-year survival of25%. The principal causes of death are myocarditisand myocardial infarction secondary to coronaryvasculitis.8 Glucocorticoid therapy has been reportedto increase the 5-year survival to more than 50%.Oral glucocorticoids in high dosage alone usually are adequate for treatmentof Chung-Strauss syndrome. Cytotoxic drugs  are necessary infewer than 20% of the cases of glucocorticoid failure.

In cyclophosphamide resistant cases plasma exchangedaily and oral etoposide are required.

Major life-threatening organ involvement may requiretreatment with pulse doses of IV corticosteroids (I/Vbolus methylprednisolone 10 mg/kg) as well as other agents.

References

1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis,and periarteritis nodosa. Am J Pathol 1951;27(2):277-301.
2. Strauss L, Churg J, Zak FG. Cutaneous lesions of allergicgranulomatosis: a histopathologic study. J Invest Dermatol1951;17(6):349-59.
3. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B,Casassus P. Churg-Strauss syndrome. Clinical study andlong-term follow up of 96 patients. Medicine (Baltimore)1999;78(1):26-37.

4. Sevinc A, Hasanoglu HC, Gokirmak M, Yildirim Z,Baysal T, Mizrak B. Allergic granulomatosis and angiitisin the absence of asthma and blood eosinophilia: a rare presentation of limited Churg-Strauss syndrome.Rheumatol Int 2004;24(5):301-4.

5. Saxena S, Joshi JM. Multiple pulmonary nodules. IndianJ Chest Dis Allied Sci 2005;47(3):193-5.
6. Masi AT, Hunder GG, Lie JT, Michel BA, Block DA,Arend WP. The American College of Rheumatology 1990criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum1990;33(8):1094-100.

7. Lie JT. Histopathologic specificity of systemic vasculitis.Rheum Dis Clin North Am 1995;21(4):883-909.
8. Eustace JA, Nadasdy T, Choi M. Disease of the month.The Churg Strauss Syndrome. J Am Soc Nephrol1999;10(9):2048-55.