An Unusual Presentation of Churg-Strauss Disease (Allergic Angiitis and Granulomatosis)


Dr A Bhatt, Dr J Jain, Dr Op Gupta, Dr N Gangane    13 November 2017

Dept. of Medicine Dept. of Pathology Mahatma Gandhi Institute of Medical Sciences Sewagram Wardha Maharashtra Abstract We present a case of a 45 year old female with history of four days of fever and rash with swelling over both limbs diagnose don postmortem autopsy as Churg Strauss disease and further give a brief overview of the disease. 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 examination was 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 Good pasture 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.6 The presence of four or more criteria yields a sensitivityof 85 and a specificity of 99.7 . These criteria are Asthma wheezing expiratory ronchi Eosinophilia of more than 10 in peripheral smear Paranasal sinusitis Pulmonary infiltrates may be transient 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.

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