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Churg-Strauss Syndrome and Revascularization of the Hand: A Case Report To the Editor: Churg-Strauss syndrome (CSS) is characterized by allergic rhinitis, asthma, and peripheral blood eosinophilia resulting from systemic vasculitis of small to medium-size vessels1. Although the exact cause of the syndrome is not known, it is felt to be due to an autoimmune process2. Lack of easy diagnosis and full understanding of the disease process makes treatment challenging. We describe a case of critical ischemia of the hand due to CSS in which a combination of medical and surgical treatment salvaged the hand. A 54-year-old man presented with severe pain and black discoloration to several digits of both hands. In the 6 months prior to the referral he had noticed worsening of new Raynaud's symptoms in both hands. His history included a 12-year period of intermittent steroid-dependent asthma (both oral and inhaled steroid), a 6-year history of sinusitis with nasal polyposis resulting in 3 separate polypectomies, and a single episode of pulmonary hemorrhage requiring hospitalization 7 years earlier. He had a significant allergy to aspirin and nonsteroidal antiinflammatory medications resulting in a severe asthmatic reaction. He was admitted to hospital with a diagnosis of critical ischemia of the second and third digits of the left hand. Left subclavian angiography was performed and the results were consistent with decreased flow through both the radial and ulnar arteries. There were occlusions of the radial artery at the level of the anatomical "snuff box" and the ulnar artery proximal to the wrist crease and extending into the superficial palmar arch (Figure 1). Nitroglycerin given intravenously for vasodilation resulted in improved but subnormal flow through both arteries. Other significant investigations showed hypereosinophilia (25% on differential of white blood cell count on more than 2 occasions), elevated immunoglobulin E to 156 g/l, elevated immunoglobulin G to 5.3 g/l, normal erythrocyte sedimentation rate and C-reactive protein, and negative antinuclear antibodies, cryoglobulins and antineutrophil cytoplasmic antibodies (both cANCA and pANCA). A computerized tomography scan of his sinuses revealed extensively thickened mucosa in both the maxillary and ethmoidal sinuses. He was given a trial infusion of 72 h of continuous intravenous alprastodil (20 µg/kg/min) without noticeable improvement. The anesthesia service was consulted and a stellate ganglion block was completed that did not provide any pain relief or improvement in symptoms.
Due to ongoing ischemia and pain he was referred to the plastic surgery service for possible surgical intervention. He underwent exploration and excision of thrombosed sections of both ulnar and radial arteries. The radial artery was reconstructed with an 8 cm interpositional vein graft at the level of the anatomical snuff box. The ulnar artery was reconstructed with a 13 cm interpositional vein graft extending from the distal one-third of the forearm to the palmar arch. A palmar digital sympathetectomy of the common digital arteries in the palm was also completed in addition to the revascularization. During the same procedure otolaryngology performed bilateral nasal polypectomy for tissue samples. Pathology specimens obtained in the operating room revealed numerous changes. The ulnar artery biopsy demonstrated a healed vasculitis within the vessel wall and an organized (7 cm long and 2–3 cm wide) thrombus with many eosinophils present (Figure 2). The radial artery demonstrated near complete obliteration of the vascular lumen by recanalized organized thrombus. Nasal specimens showed pathological evidence of inflammatory polyps with absence of vasculitis.
Postoperatively the patient had complete resolution of pain and ischemic signs and symptoms in his left hand. At followup a year later he was doing remarkably well and his vasculitis continued to be in remission. His medications had been reduced to include azathioprine 100 mg orally daily, prednisone 5 mg daily equivalent dose, nifedipine 20 mg orally daily, and clopidogrel 75 mg orally daily. He had regained his sense of smell, which had been absent for more than 9 years. Both radial and ulnar pulses were palpable with no pain or symptoms. The function of his left hand was essentially normal with a normal Allen's test. CSS is classified as a small-vessel vasculitis. In the last 15 years there have been only 4 reported cases of CSS complicated by forearm vessel occlusion3. This is the first case of Churg-Strauss vasculitis with occlusion of both the radial and ulnar arteries in which surgical revascularization was used to avoid amputation. Although CSS is relatively rare and digital necrosis is uncommon, when critical ischemia occurs this frequently leads to amputation. The presence of thrombosis in the absence of ANCA positivity and other thrombophilic factors has been reported in both hypereosinophilic syndrome and CSS3-6. Our patient did not demonstrate elevated inflammatory markers at the time of presentation, but his pathology revealed healing vasculitis and active clot formation at the time of surgical revascularization. This made it extremely difficult to determine whether vasculitis, thrombosis, or the combination of the 2 were present. Clinically, he responded only when treated with a combination of medical and surgical therapy. Raynaud's and digital necrosis have been documented in 3 cohorts of patients with CSS at a rate of 1%–6%7-9. Many other studies document generalized cutaneous changes in large categories but do not clearly indicate if digital necrosis is involved. In many cases angiography is not carried out and the extent of digital ischemia is determined clinically. In some cases with digital ischemia, medical therapy was sufficient to reverse the ischemia and avoid surgical intervention5,10. However, there are several reported cases of digital necrosis in CSS in which the patients progress to amputation despite treatment3,8,11,12. We have described a patient with CSS who developed critical digital ischemia that failed all medical therapies for revascularization. The combination of surgical and postsurgical medical therapy allowed return of normal function of this patient's hand. ANNIKA CARD, MD, Division of Plastic Surgery, Department of Surgery; NADER KHALIDI, MD, Section of Rheumatology, McMaster University, Hamilton; MARIE CLEMENTS-BAKER, MD, Department of Medicine, Queen's University, Kingston; ACHILLES THOMA, MD, Division of Plastic Surgery, Department of Surgery, McMaster University, 101-206 James St. South, Hamilton, Ontario L8P 3A9, Canada. Address reprint requests to Dr. Thoma. E-mail: athoma@mcmaster.ca 2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-92. [MEDLINE] 3. Otani Y, Anzai S, Shibuya H, et al. Churg-Strauss syndrome manifested as necrosis of fingers and toes and liver infarction. J Dermatol 2003:30:810-5. [MEDLINE] 4. Inui S, Itami S, Iwai C, Yoshikawa K. Churg-Strauss syndrome involving medium-sized arteries. J Dermatol 2001;28:161-4. [MEDLINE] 5. Oppliger R, Gay-Crosier F, Dayert E, Hauser C. Digital necrosis in a patient with hypereosinophilic syndrome in the absence of cutaneous eosinophilic vasculitis. Br J Dermatol 2001;144:1087. [MEDLINE] 6. Hachulla E, Hatron PY, Janin A, Robert Y, Devulder B. Digital arteries, thrombosis and hypereosinophilic syndrome: an uncommon complication [French]. Rev Med Interne 1995; 16:434-6. [MEDLINE] 7. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: Clinical study and long-term follow-up of 96 patients. Medicine 1999;78:26-37. [MEDLINE] 8. Jang K, Lim Y-S, Choi J-H, Sung KJ, Moon KC, Koh JK. Hypereosinophilic syndrome presenting as cutaneous necrotizing vasculitis and Raynaud's phenomenon complicated by digital gangrene. Br J Dermatol 2000;143:641-4. [MEDLINE] 9. Mouthon L, Le Toumelin P, Andre MH, Gayraud M, Casassus P, Guillevin L. Polyarteritis nodosa and Churg-Strauss angiitis characteristics and outcome in 38 patients over 65. Medicine 2002;81:27-40. [MEDLINE] 10. Mouthon L, Khaled M, Cohen P, Guillevin L, Mouthon L, Subra JF. Systemic small sized vessel vasculitis after massive antigen inhalation. Ann Rheum Dis 2001;60:903-4. [MEDLINE] 11. Sharma BK, Daga MK, Sharma M. A limited form of Churg-Strauss syndrome presenting without asthma and eosinophilia. Med J Aust 2004;181:498-9. [MEDLINE] 12. Liozon E, Delaire L, Lacroix P, et al. Raynaud syndrome complicated by digital gangrene during treatment with interferon-alpha [French]. Rev Med Interne 1997;18:316-9. [MEDLINE]
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