Churg-Strauss Syndrome :: complications
Acute Med. 2012 ;11 (2):89-92 22685699
John Radcliffe Hospital, Headley way, Headington, Oxford, OX3 9DU, UK.
Churg-Strauss syndrome is an uncommon multisystem disease involving small and medium-sized arteries, capillaries, veins, and venules. It is characterised by the presence of asthma, hypereosinophilia, and evidence of vasculitis affecting a number of organs. It is important for acute medical physicians to recognise this condition as delayed diagnosis results in a higher morbidity and mortality. Here we describe a case of Churg-Strauss syndrome in a 41 year old woman presenting with a recurrent painful rash. The clinical manifestations and management of this condition are discussed.
Most cited papers:
The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
A T Masi, G G Hunder, J T Lie, B A Michel, D A Bloch, W P Arend, L H Calabrese, S M Edworthy, A S Fauci, R Y Leavitt
University of Illinois College of Medicine, Peoria.
Criteria for the classification of Churg-Strauss syndrome (CSS) were developed by comparing 20 patients who had this diagnosis with 787 control patients with other forms of vasculitis. For the traditional format classification, 6 criteria were selected: asthma, eosinophilia greater than 10% on differential white blood cell count, mononeuropathy (including multiplex) or polyneuropathy, non-fixed pulmonary infiltrates on roentgenography, paranasal sinus abnormality, and biopsy containing a blood vessel with extravascular eosinophils. The presence of 4 or more of these 6 criteria yielded a sensitivity of 85% and a specificity of 99.7%. A classification tree was also constructed with 3 selected criteria: asthma, eosinophilia greater than 10% on differential white blood cell count, and history of documented allergy other than asthma or drug sensitivity. If a subject has eosinophilia and a documented history of either asthma or allergy, then that subject is classified as having CSS. For the tree classification, the sensitivity was 95% and the specificity was 99.2%. Advantages of the traditional format compared with the classification tree format, when applied to patients with systemic vasculitis, and their comparison with earlier work on CSS are discussed.
Department of Internal Medicine, Hôpital Avicenne, Université Paris-Nord, Bobigny, France.
Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.
Hôpital Delafontaine, Saint-Denis, France.
Polyarteritis nodosa (PAN), first described by Küssmaul and Maier, is a well-known form of necrotizing angiitis whose manifestations are weight loss, fever, asthenia, peripheral neuropathy, renal involvement, musculoskeletal and cutaneous manifestations, hypertension, gastrointestinal tract involvement, and cardiac failure. Recently individualized from PAN, microscopic polyangiitis (MPA) is a systemic vasculitis of small-size vessels whose clinical manifestations are very similar to those of PAN, but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Churg Strauss syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis similar to that of PAN and occurring in individuals with asthma and allergic rhinitis. Considering the etiologies of PAN, primary and secondary vasculitides can also be distinguished because PAN can be the consequence of hepatitis B virus (HBV) infection and sometimes of other etiologic agents. The prognosis of systemic vasculitides has been transformed by corticosteroids that are, except in HBV-related PAN, the basic treatment. Immunosuppressive drugs, especially cyclophophamide, have also contributed to improving the prognosis, but their precise role in the management of these vasculitides is still being elucidated.
R Solans, J A Bosch, C Pérez-Bocanegra, A Selva, P Huguet, J Alijotas, R Orriols, L Armadans, M Vilardell
Department of Internal Medicine, Department of Pathology, Department of Pneumology and. Department of Preventive Medicine, Vall d'Hebrón University General Hospital, 08035 Barcelona, Spain.
OBJECTIVES To study the clinical spectrum and evolution of Churg-Strauss syndrome in order to assess the clinicopathological features of the disease, the response to treatment and the long-term outcome. METHODS Thirty-two patients with proven allergic and granulomatous angiitis (Churg-Strauss syndrome) and followed up at a single institution were evaluated. They were recruited between 1977 and 1999 from internal medicine departments. Data were obtained retrospectively from medical files in 15 cases and prospectively, using a standardized form, for the remaining patients. RESULTS All patients had asthma and hypereosinophilia. The lungs, skin and peripheral nervous system were the organs most frequently involved. Antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity (MPO-ANCA) were detected in 77.8% of tested patients but they were not useful for monitoring disease activity. Extravascular granulomas were rarely seen in tissue biopsies. Forty per cent of the patients were treated with steroids alone. Immunosuppressive agents were added to the treatment when severe neurological, cardiac or gastrointestinal involvement was present. The outcome and long-term survival were good. Clinical relapse was rare after the first year of therapy. Dysaesthesiae of the distal limbs, neurophatic pain and cardiac failure were the most frequent sequelae. CONCLUSIONS Churg-Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, skin and peripheral nervous system. Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.
Department of Neurology, Nagoya University School of Medicine, Tsurumai, Japan.
We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. Initial symptoms attributable to neuropathy were acute painful dysaesthesiae and oedema in the dysaesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a pattern of mononeuritis multiplex in the initial phase, progressing into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated and unmyelinated fibres due to axonal degeneration was evident as decreased or absent amplitudes of sensory nerve action potentials and compound muscle action potentials, indicating acute massive axonal loss. Epineurial necrotizing vasculitis was seen in 54% of cases; infiltrates consisted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbers than lymphocytes. CD20-positive B lymphocytes were seen only occasionally. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in a single patient, indicating a good survival rate. The patients who responded well to the initial corticosteroid therapy within 4 weeks regained self-controlled functional status in longterm follow-up (modified Rankin score was < or = 2), while those not responding well to the initial corticosteroid therapy led a dependent existence (P < 0.01). In addition the patients with poor functional outcomes had significantly more systemic organ damage caused by vasculitis (P < 0.05). Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis.
Mayo Medical School, Mayo Clinic Rochester, Minnesota.
OBJECTIVE: To determine the frequency and the types of neurologic involvement in a series of patients with Churg-Strauss syndrome (CSS). DESIGN: We reviewed the medical records of 47 consecutive patients with CSS who were examined at the Mayo Clinic between January 1974 and June 1992. MATERIAL AND METHODS: The study patients were classified into two groups:(1) those with a histopathologically confirmed diagnosis of CSS who had evidence of either vasculitis or Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 33) and (2) those with a clinical diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesions, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 14). RESULTS: Of the 47 patients, 29 (62%) had neurologic involvement. Peripheral neuropathy was detected in 25 patients: 17 had multiple mononeuropathy, 7 had distal symmetric polyneuropathy, and 1 had an asymmetric polyneuropathy. Three patients had cerebral infarctions. Less commonly identified problems included radiculopathies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration, 6.7 years. Follow-up data, when available, showed that corticosteroid therapy usually yielded improvement or stabilization. CONCLUSION: Neurologic involvement is common in CSS, usually manifesting as peripheral neuropathy. In this series of patients, asthma preceded the neurologic manifestations.
Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.
Renato A Sinico, Lucafrancesco Di Toma, Umberto Maggiore, Paolo Bottero, Antonella Radice, Cinzia Tosoni, Chiara Grasselli, Laura Pavone, Gina Gregorini, Stefano Monti, Micol Frassi, Filomena Vecchio, Caterina Corace, Emanuela Venegoni, Carlo Buzio
Dipartimento di Nefrologia e Immunologia, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milan, Italy. email@example.com
OBJECTIVE Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features. METHODS Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status. RESULTS ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042). CONCLUSION ANCAs are present in approximately 40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.
Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: indications for the classification of vasculitides of the polyarteritis Nodosa Group.
Service de Médecine Interne, Hôpital Avicenne, Bobigny, France.
The present study attempted to define the clinical, radiological, immunological and pathological characteristics of microscopic polyangiitis (MPA), and to separate them from classic PAN (c-PAN) and Churg-Strauss syndrome (CSS). In most cases, patients presenting microaneurysms and/or multiple vessel stenoses, which reflect medium-sized vessel involvement, did not have antineutrophil cytoplasmic antibodies (ANCA)(6.6%). Conversely, patients with glomerulonephritis almost never had abnormal angiograms. Furthermore, the clinical characteristics of ANCA-positive patients also indicate small-sized vessel involvement. Skin involvement (73.1 vs 26.7%, P < or = 0.05), glomerulonephritis (38.5 vs 0%, P < or = 0.001) and the presence of ANCA (34.6 vs 6.7%, P < or = 0.05) were significantly more frequent in patients with normal than abnormal angiograms, respectively. Conversely, hypertension (66.7 vs 23.1%, P < or = 0.02), renal vasculitis (46.7 vs 0%, P < or = 0.001) and hepatitis B antigenaemia (60 vs 11.5%, P < or = 0.01) were significantly more common in patients with abnormal angiograms. Stratification of patients according to vessel size showed that, except for skin involvement (P < or = 0.05) and glomerulonephritis (P < or = 0.01), which are direct manifestations of small-sized vessel diseases, clinical symptoms of PAN or CSS, angiographic findings and ANCA were not correlated to arteriole size. Although at present it is not possible to separate definitively MPA from c-PAN, our results show that ANCA should be considered diagnostic for MPA and, in most cases, should be an exclusion criterion for c-PAN. Conversely, small-sized vessel involvement can be observed in patients presenting characteristics of c-PAN, MPA or CSS and, therefore, is not a sufficient criterion for assigning diagnosis.
Division of Nephrology and Hypertension, University of North Carolina, Chapel Hill 27599-7155, USA. firstname.lastname@example.org
BACKGROUND Unlike Goodpasture's syndrome with diffuse alveolar hemorrhage (DAH), there are few studies examining therapy for patients with DAH associated with antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (SVV). METHODS We performed a retrospective review of all such patients presenting to our institution between 1995 and 2001. All patients were treated with apheresis and induction immunosuppressive therapy; namely, intravenous methylprednisolone and/or intravenous cyclophosphamide. RESULTS DAH resolved with apheresis in 20 of 20 patients (100%) with 6.4 (average) treatments. There were no complications of therapy. Half the patients (7 of 14) who also presented with azotemia were discharged with improved renal function. CONCLUSION Patients with ANCA-related SVV and DAH benefit from prompt initiation of apheresis coupled with aggressive immunosuppressive therapy. Such therapy can be lifesaving with respect to the pulmonary component of this syndrome.
Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome--62 patients.
OBJECTIVE. Antibodies directed against components of neutrophil cytoplasm have been detected in various systemic vasculitides and especially in Wegener's granulomatosis. In polyarteritis nodosa (PAN) and Churg-Strauss syndrome, few data are available and correlation between clinical manifestations and antineutrophil cytoplasm antibodies (ANCA) has not been established. Therefore, we tested, before treatment of vasculitis, 62 consecutive patients suffering from PAN with hepatitis B virus (HBV) markers, PAN of unknown etiology or Churg-Strauss syndrome. METHODS. Only patients with PAN and Churg-Strauss syndrome were included in the study. The diseases were histologically and/or angiographically proven. Every patient's serum was tested by an indirect immunofluorescence assay (IFA) and, in 37 cases, by an enzyme linked immunosorbent assay (ELISA). RESULTS. ANCA detected by IFA were observed in 10.7% of the patients with PAN with HBV markers, in 27.3% of the patients with PAN without HBV markers and in 66.7% of the patients with Churg-Strauss syndrome. When ELISA was performed, 11.1% of the patients with PAN associated with HBV infection, 20% of the patients with PAN without HBV markers and 55.6% of the patients with Churg-Strauss syndrome were positive. ANCA were positively correlated with asthma and purpura and negatively correlated with HBV markers. CONCLUSION. Regardless of the technique used, Churg-Strauss syndrome was associated with ANCA in about 60% of the cases while, in PAN of unknown etiology, ANCA were found in about 25% of cases. In contrast, IFA and ELISA only detected ANCA in a limited number of cases of PAN related to HBV infection. ELISA positivity in patients with PAN and Churg-Strauss syndrome was usually associated with antimyeloperoxidase antibodies. In our cases of PAN, ANCA and purpura were significantly correlated, suggesting that, in these cases, small vessels are involved and therefore macroscopic and microscopic PAN coexist. Thus it seems that ANCA are essentially present in the cases of small vessel vasculitis, as has been described, and are not a marker of pure macroscopic PAN, at least at our present level of understanding of these antibodies.