Hassan, Imran

Jafri, Wasim

Khan, Javaid A.

Khan, Shaista

Pervez, Shahid

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eCommons@AKUDepartment of Pathology and Laboratory Medicine Medical College, PakistanMay 1996Churg-Strauss Syndrome with IntestinalperforatIonJavaid A. KhanAga Khan UniversityImran HassanAga Khan UniversityShahid PervezAga Khan University, shahid.pervez@aku.eduWasim JafriAga Khan UniversityShaista KhanAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiolPart of the Medical Pathology Commons, Otolaryngology Commons, Pathology Commons, andthe Respiratory Tract Diseases CommonsRecommended CitationKhan, J. A., Hassan, I., Pervez, S., Jafri, W., Khan, S. (1996). Churg-Strauss Syndrome with Intestinal perforatIon. Journal of PakistanMedical Association, 46(5), 109-110.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol/308Churg-Strauss Syndrome with Intestinal PerforatIonPages with reference to book, From 109 To 110 Javaid A. Khan, Imran Hassan ( Departments of Medicine, The Aga Khan University Hospital, Karachi. ) Shahid Pervaiz ( Department of Pathology, The Aga Khan University Hospital, Karachi. ) Wasim Jafri ( Department of Medicine, The Aga Khan University Hospital, Karachi. ) Shaista Khan ( Departments of Surgery, The Aga Khan University Hospital, Karachi. ) Introduction Churg Strauss syndrome (CSS) (allergic granulomatosis and angiitis) is a systemic disordercharacterized by asthma, transient pulmonaiy infiltrates, hypereosinophilia and a systemic vasculitis1-3.CSS is a rare disease; however, this syndmme has been reported with increasing frequency in recentyears2. The organs most evidently involved clinically are the heart, lungs, kidney, gut, skin andperipheral nerves3.We present a case of Churg-Strauss Syndrome complicated by intestinal perforation and intracerebralhemorrhage, alongwith a review of literature regarding gastrointestinal involvement in this condition.Case History A 45 years old gentleman was admitted to the hospital beóause of headache, dysphasia and righthemiparesis for one day. He had been well until two years ago when he began experiencing paroxysmalattacks of wheezing. A total white count done at that time was 18x109/L with 73% eosinophils. He wasprescribed inhaledbmnchodilators and steroids,yet he continued to have episodes of breathlessness andwheezing during the next two years.One month prior to admission to the hospital he began complainingof intensejoint pain, dyspnoea and weakness in his hands. He also developed a papular rash over bothfeet A complete blood picture showed the white blood count to be 30.9x109/L with 64% eosinophils. Askin biopsy was taken which showed leukoclastic vasculitis (Figure).He was treated with prednisolone for one week following which his symptoms, particularly hiswheezing improved.At the time of admission, he was conscious but disorientated in time, place and person. He wasafebrile, pulse was 90/minute and blood pressure was 140/80 mmHg. Neurological examinationshoweddysphasia and upper motor neuron palsy of the seventh and twelfth cranial nerves on the right side.There was right hemiparesis alongwith bilateral wrist drop with wasting of small muscles of bothhands. There also was evidence of Raynaud’s phenomenon. Examination of all other systems wasunremarkable. His hemoglobin was 14.4 g/dl and white cell count was 9x109fL with 70% polymorphs,18% lymphocytes and 12%eosinophils. A CT scan showed an intracerebral bleed in the left temporalregion. Subsequently his neurological status improved over the next few days. The nerve conductionstudy was consistent with mononeuritis multiplex. His anti-neutrophilic cytoplasmic antibodies(ANCA) was positive while the chest x-ray was normal. He was treated with cyclophosphaniide andsteroids.On the sixth day of admission, he suddenly developed severe abdominal pain which graduallyincreased. He became hypotensive and developed signs of generalized peritonitis. An X-ray film of theabdomen showed pneumoperitoneum. A laparotomy was performed and per operatively a solitaiypunched out perforation 135 cm from the duodenojejunal flexure was seen. Wedge resection andprimary closure of the perforation was done.Histopathology of the gut wall taken from the perforation showed heavy infiltration of acuteinflammatory cells.His post-operative course was uneventful and he was discharged on Predmsolone andCyclophosphamide. Six months post-operation, he had a grand-mal seizure, while coming back fromwork and was started on Phenytoin. Till the writing of this report it has been over 2 years since theoperation and he has remained asymptomatic on 5 mg Prednisolone.Discussion The original description of this disorder as a distinct clinicopathological entity was done by Churg andStrauss in 19492,3. Since then, several reports of CSS have appeared in the literature. Although Lanhamet al1 reviewed 154 cases with CSS in 1984, this remains a rare disease.Lanham et al noted 3 criteria for the diagnosis of CSS; asthma peak peripheral blood eosinophil countin excess of 1 .5x109/L and the characteristic systemic vasculitis involving 2 or more extrapulmonaryorgans. The presentation of this syndrome is highly variable and its clinical manifestations are protean5.CSS can involve a number of organs including the cardiovascular, pulmonary, gastrointestinal, nervous,renal and musculoskeletal system6,7. Cardiac disease is recorded as the major cause of death in CSS. Itcan present as pericarditis, cardiac failure or merely an abnormal electrocardiogram. Pulmonaiy diseasemay manifest as infiltrates which are generally transient. Pulmonary hemorrhage is an importantcomplication. Pleural effusion can occur and contains large amount ofeosinophils. Renal disease occursin the majority of the patients and is characterized by focal segmental glomerulonephritis, but renalfailure is relativ ely uncommon. The usual lesion in CNS involvement is a mononeuritis multiplex.although a diffuse symmetric neuropathy can occur. Cerebral infarction, although rare is the secondmost common cause of death. Rash is a common feature, appearing as palpable purpura, erythemaorurticaria. Arthritis orarthralgia may also be seen.Our patient demonstrates several important features of CSS. He initially had adult onset of asthma andperipheral eosinophilia. Later on he had evidence of a systemic disease involving the joints, skin,central nervous system and gastrointestinal tract.Gastrointestinal symptoms in CSS include abdominal pain, bloody stools, diarrhoea and nausea andvomiting. Although gastrointestinal symptoms are commonly seen, the literature contains few detaileddescriptions of the gastrointestinal lesion2. The gastrointestinal tract may be involved in 20% of thepatients with CSS and typically results in intestinal inflammation, ulceration, perforation orhemorrhage8.In a review of 154 cases reported in the English literature, Lanharn found abdominal pain as a commonsymptom (59% of all cases) which reflected bowel perforation, peritonitis, intestinal obstruction,mesenteric vasculitis or cholecystitis6. In a large series of 165 patients which included both ChurgStrauss syndrome and Polyarterities Nodosa patients9, 52 (31%) had abdominal signs and symptoms.The most severe episodes of abdominal pain were due to peritonitis (9 cases), duodenal ulcer (3 cases),intestinal infarction (1 case) and unexplained pain (1 case). while 11 patients presented withgastrointestinal hemorrhage. In the Japanese literature there have been 21 cases of lesions in the gastro-intestinal tract in association with CSS. These lesions were in the form of multiple ulcers located in thestomach, small intestine and the colon. Twelve of these 21 cases presented as a gastrointestinalperforation. The small intestine was the most common site of involvement and the most frequentlyperforated. Most of the cases in the literature were not diagnosed until surgery or death2.Other gastrointestinal manifestations of CSS reported in the literature are chronic active hepatitis,chronic ulcerative colitis10, intrahepatic duct perforation caused by hepatic aneurysm rupture11 and acalculous cholecystitis12. Schoretsantis et al have recently reported a case of Churg-Strauss syndromewhere severe mesenteric intestinal ischemia lead to small bowel necrosis13. This patient underwent fivelaparotomies for resection of small bowel and survived.The case that we have described fits most likely to the diagnosis of Churg Strauss Syndrome (CSS).Abdominal pain and intestinal ulceration and perforation are not rare. If physicians and surgeons keepthis condition in mind and accurate and reliable histopathological reports are available, we have nodoubt that more of such cases would be diagnosed.In tropical countries eosinophilia should not belabeled as only due to parasitic and allergic causes. Other less common causes like Churg strausssyndrome and Polyarteritis Nodosa should also be considered.Acknowledgement We are grateful to Mr. Sulaiman S. Gilani for his help in the preparation of this manuscript.References 1. Vogel, P.S., Nemer, J., Sau. P. et al. Churg Strauss syndrome J. Am. Acad. Dermatol.. 1992;5 822-824.2. Shimamoto, C., Hirata, I., Ohshiba, S. et at. Churg-Strauss syndrome (allergic granulomatousangiitis) with peculiar multiple colonic ulcers. Am. 3. Gastroen. terol., 1990,55.316-19.3. Schwartz, R.A. and Churg, J. Churg-Strauss syndrome. British J. Dermatol., 1992:127:199-204.4. Lanham, JO. Churg-Strauss syndrome. Br. J. Hosp. Med.. 1992;47:667.673.5. Hasley, PB., Follansbee, W.P. and Coulehan, J.L. Cardiac manifestations of Churg-Strauss syndrome:Report of a case and review of the literature. Am. Heart J., 1990;120:996-999.6. Lanham. J.G., Elkon, KB, Pusey, CD. Systemic vasculitis with asthma and cosinophilia: A clinicalapproach to the Churg.Strauss syndrome. Medicine, 1984;63.2:65-81.7. Chum,bley, L.C.. Harrison. E.G. and DeRemee, R.A. Allergic Granulomatosis and angirtis(Churg.Strauss syndrome), Mayo. Clin. Proc., 1977;52:477-484.8. Camilleri, M . Pusey. C.D.. Chadwick, VS. et al. Gastrointestinal manifestation ofsystemic vasculitis.Q. J. Med., 1983; 141-149.9. Guilleuin, L., Huong, D.L.T, Gorden, P. etal. Clinical findings and prognosis of polyarterisis nodosaand churg-strauss angitis: A study of 165 patients. Br. 3. Rheumatol., 1988;27:258-264.10. Finan, MC. and Winkkmann, R.K. The cutaneous extravascular necrotizing granuloma (Churg-Strauss Granuloma) and systemic disease: A review of 27 cases. Medicine, 1983;62: 3:142-157.11. Imai, H., Nakamoto, Y., Nakajima, Y. et at. Allergic granulomatosis and angiitis (Churg.Strausssyndrome) presenting as a calculous cholecystitis. 3. Rheumatot., 1990;17:247-9.12. Nakamura. S., Yokoi, Y., Suzuki, S. et at. A case of melena caused by a hepatic aneurysm rupturedinto the intrahepatic bile duct in a patient with allergic granulomatous angiitis. Jpn. J. Surg.,1991;21471-475.13.Schoretsanitis, G.N , Wakely, D.M., Maddox, T. et at. A case of Churg-Strauss vasculitiscomplicated by small bowel necrosis. Postgrad. Med,J., 1993;69:828-831.

Churg-Strauss Syndrome with Intestinal perforatIon

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Churg-Strauss Syndrome with Intestinal perforatIon

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