Giovanni Garini

Ear, nose and throat manifestations of Churg-Strauss syndrome

Conclusion. Ear, nose and throat (ENT) involvement is common in Churg-Strauss syndrome (CSS), usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps. Otolaryngologists may play a pivotal role in making an early diagnosis of this disease. Objectives. CSS is a systemic vasculitic disorder that affects small to medium-sized blood vessels. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by activated eosinophils. Patients affected by CSS frequently have ENT manifestations, which are often present at the time of disease onset and may represent relevant clues for the diagnosis. Thus, our objective was to present the ENT manifestations at the onset, at the diagnosis and at some point during the course of the disease in a series of patients with CSS collected at a single center. Materials and methods. Twenty-eight patients with CSS, as defined according to the 1990 American College of Rheumatology classification criteria, were identified. Twenty-one (75%) of these patients had ENT involvement. We evaluated the clinical course, laboratory data, histologic findings, treatment and outcomes. Results. Of the 21 patients, 13 (61.9%) had ENT involvement at asthma onset and 8 (38%) at diagnosis or during follow-up. The most common ENT manifestations were allergic rhinitis in 9 (42.8%) patients and nasal polyposis in 16 (76.1%). Three (14.2%) patients developed chronic rhinosinusitis without polyps, three (14.2%) had nasal crusting, one (4.7%) serous otitis media, one (4.7%) purulent otitis media, two (9.5%) progressive sensorineural hearing loss, and one (4.7%) unilateral facial palsy. Corticosteroid therapy associated with immunosuppressive drugs usually yielded improvement or stabilization.

Large bowel obstruction heralding Churg-Strauss syndrome

TO THE EDITOR: Churg-Strauss syndrome (CSS) is characterized by asthma, hypereosinophilia, necrotizing vasculitis, and extravascular granulomas. Other manifestations include mononeuritis multiplex, lung infiltrates, skin signs, and gastrointestinal (GI) tract involvement (1). A 60-year-old woman was admitted because of abdominal pain, asthenia, and low-grade fever (37.6◦C). Physical examination revealed diffuse abdominal tenderness. Laboratory tests showed a white blood cell count of 8280/mm3, with 33% eosinophils. The erythrocyte sedimentation rate (ESR) was 60 mm/Ih and the level of C-reactive protein (CRP) was 25.9 mg/L (normal <5 mg/L). Total serum IgE were markedly high (9780 IU/ml, normal range 1–150 IU/ml) and radioallergosorbent testing revealed the presence of specific IgE to cat. Antinuclear antibodies were positive (titer 1/160, “nucleolar” pattern), but anti-dsDNA, anti-extractable nuclear antigen, and antineutrophil cytoplasmic antibodies (ANCA) were negative. A chest computer tomography (CT) was normal, but a plain abdominal X-ray showed a distended small intestine with air-fluid levels; finally, a barium enema revealed a stenotic lesion with an “apple core” appearance involving the ascending colon (Fig. 1A). A laparotomy was performed, and the terminal ileum and the ascending colon were resected. Gross pathological examination confirmed the presence of a 5-cm long circumferential stenotic lesion; microscopic examination revealed an intense eosinophil-rich inflammatory infiltrate throughout the intestinal wall but mainly in the submucosal layer, together with necrotizing vasculitis of small vessels (Fig. 1B), and eosinophilic granulomas (Fig. 1C). The mucosa was also involved, with gland destruction and crypt eosinophilic abscesses (Fig. 1D). A few days after the operation, multiple skin nodules and urticarial lesions appeared, and biopsy of one of the nodules showed leukocytoclastic vasculitis. CSS was diagnosed, and oral treatment with prednisone and cyclophosphamide was started, with prompt resolution of the skin lesions and normalization of the eosinophil count, ESR, and CRP. Immunosuppressive therapy was stopped after 1 yr but, 3 months later, CSS relapsed with lung involvement: a chest CT revealed two nodules in the left lung and a “ground-glass” appearance of the surrounding lung parenchyma. Immunosuppressive treatment was resumed and 5 months later there were no signs of active disease. CSS involves the GI tract in about one third of the patients; in most cases, it affects the stomach and the small bowel, which are injured as a result of vasculitis of small mesenteric blood vessels and eosinophilic infiltration of the intestinal wall (1, 2). The large bowel is rarely involved and most of the reported cases have features of ischemic colitis (3). The main clinical feature of our patient at disease onset was large bowel obstruction, which, to the best of our knowledge, has not been previously reported. The histological findings of eosinophilic tissue infiltration, granulomas, and small-vessel necrotizing vasculitis were paradigmatic of CSS (1). Eosinophilic infiltration of the colon is also characteristic of eosinophilic colitis, but the disease does not usually lead to granulomas or vasculitis (4, 5). Ulcerative colitis and Crohn’s disease often show tissue eosinophilia and granulomas, but eosinophilic infiltration is less marked than in CSS and intestinal vasculitis is rare (4, 5). Polyarteritis nodosa often involves the GI tract but seldom shows severe tissue eosinophilia and, as it usually affects medium-sized vessels, it causes ischemic alterations in large intestinal segments (6). Wegener’s granulomatosis, a systemic necrotizing vasculitis, sometimes presents with marked eosinophilia and can thus mimic CSS; in our patient, both the absence of ANCA and the clinical history of allergy made CSS a more likely diagnosis. As the clinical picture of our patient at onset lacked some of the pivotal features of CSS (e.g., asthma, peripheral neuropathy), it could not be classified as classical CSS on the basis of the American College of Rheumatology criteria (7). However, although CSS is usually considered a systemic disease, some patients present with “limited forms of CSS” (eosinophilic vasculitis and/or extravascular granulomas in isolated organs or tissues). The diagnosis of limited CSS does not preclude the possibility of a disease continuum: such as it occurs in other vasculitic syndromes (e.g., Wegener’s granulomatosis), also CSS may in time progress from a limited to a disseminated disease (8). In our patient CSS initially only affected the large bowel, but subsequently also involved the skin and lung. The present case extends the clinical spectrum of CSS to large bowel obstruction, and also highlights the possibility that, although CSS may be limited to a single organ at onset, it can subsequently become systemic.

Pathophysiology of portal hypertension and mechanisms of sodium and water retention in cirrhosis

Portal hypertension is caused by an increased resistance to portal outflow and an increased portal blood inflow. Portal hypertension is associated with an abnormal distribution of the blood volume, which is increased in the splanchnic territory and reduced in the non-splanchnic compartments. The relative underfilling of the arterial circulation is responsible for the sodium and water retention, which is a consequence of the baroceptor-mediated activation of vasoconstrictor and antinatriuretic factors triggered to restore circulatory integrity.