Churg-Strauss syndrome: a case report.

A fifty-year-old female presented with a one month history of progressive dyspnea, productive cough, pain of elbows and knees, and 40°C fever despite antibiotic treatment. She has been diagnosed of bronchial asthma over 25 years before admission and oral and depot glucocorticosteroids as a long-term therapy was applied. Recently, an attempt of inhaled corticosteroids and LABA treatment was introduced with no success. Four years before admission she also developed peripheral neuropathy. Physical examination revealed tachypnea, wheezes, rhonchi and wet cracles on auscultation, tachy?cardia, skin nodules, urticarial rash and necrotic bullae all over the body. Chest X-ray showed transient, patchy, nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone. Obstruction was found on spirometry. Tachy?cardia on ECG and myocardial fluid on ECHO were also detected. Lab exams revealed elevated CRP, WBC, eosinophils, and IgE levels. ANA and ANCA antibodies were not found. Patient was diagnosed of Churg Strauss Syndrome and initial treatment of prednisone was introduced. After four days of treatment, temperature normalized, and dyspnea diminished. After one month of therapy skin lesions regressed. After 18 months of the treatment patient reports no signs, nor symptoms of the disease. Patient continues oral corticosteroid therapy.

Introduction

Churg-Strauss syndrome (CSS) so called allergic granulomatosis is a rare primary, non-inheritable, non-transmissible systemic disease, which typically occurs in patients with a history of bronchial asthma. Peripheral eosinophilia and medium and small vessel autoimmune necrotizing vasculitis, involving multiple organs are the main features of the disease [1]. CSS can be diagnosed by the presence of any four or more of the six criteria, including asthma, eosinophilia greater than 10%, paranasal sinusitis, pulmonary infiltration, histologically confirmed vasculitis and neuropathy [1].

Case Report

A fifty-year-old female presented with a one month history of progressive dyspnea, productive cough, pain of elbows and knees, and 40°C fever despite antibiotic treatment. She had been diagnosed with bronchial asthma over 25 years before admission and oral and depot glucocorticosteroids as a long-term therapy was applied. Recently an attempt of inhaled corticosteroids and LABA treatment was introduced with no success. Four years before admission she also developed peripheral neuropathy. Physical examination revealed tachypnea, wheezes, rhonchi and wet cracles on auscultation, tachycardia, skin nodules, urticarial rash and necrotic bullae all over the body (Table 1, Figure 1).

Table 1

Principal clinical, radiologic and laboratory features of the case.

Organ/method of detection	Features
Skin	Skin nodules, urticarial rash, necrotic bullae
Lung auscultation	Tachypnea, wheezes, rhonchi, wet cracles
Chest X-ray	Transient, patchy, nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone
ECG/ECHO	Tachycardia/myocardial fluid
Spirometry	FEV1
Skin biopsy	Focal, necrotizing vasculitis
Transbronchial lung biopsy	Granulomas, formed by eosynophils with central necrosis, macrophages and giant cells
Sedimentation 1 h/2 h	90/100
CRP	122 mg/l
WBC	46 × 106
Eosynophils	28 × 106 - 62%
IgE total	287.4 IU
ANA/ANCA	-/-

Figure 1

Skin lesions. Skin nodules, urticarial rash and necrotic bullae (A and B).

Chest X-ray showed transient, patchy, nonsegmental areas of consolidation, with predilection for lower zones with the area of consolidation in lower left zone (Table 1, Figure 2A). Tachycardia on ECG and myocardial fluid on ECHO were also found. Obstruction was found on spirometry. Skin biopsy revealed focal, necrotizing vasculitis. Transbronchial lung biopsy revealed granulomas, formed by eosynophils with central necrosis, macrophages and giant cells (Table 1, Figure 2B). Lab exams revealed elevated CRP, WBC, eosinophils and IgE levels. ANA and ANCA antibodies were not found.

Figure 2

A - X-ray of the chest: transient, patchy, nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone; B-Transbronchial lung biopsy: granulomas, formed by eosynophils with central necrosis, macro phages and giant cells.

Patient was diagnosed with Churg Strauss Syndrome and initial treatment with prednisone was introduced at a dose of 60 mg per day, i.e. 1 mg/kg/day. After four days of treatment, temperature normalized, and dyspnea diminished. After one month of therapy, skin lesions regressed. Then, prednisone dose was titrated according to the level of blood eosinophils. After 18 months of treatment, the patient reported no signs or symptoms of the disease. Patient continues oral corticosteroid therapy at a dose of 10 mg of prednisone per day.

Discussion

Idiopathic primary systemic vasculitides are defined, according to the Chapel Hill Consensus Conference [2], on the basis of the size of vessels involved, clinical symptoms, and histopathological features. According to the American College of Rheumatology (ACR), classification criteria of the CSS are: small vessel vasculitis along with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and idiopathic necrotizing crescentic glomerulonephritis NCGN [3]. Although, CSS can affect any organ system in the body, lung blood vessels involvement is one of the main features of the disease. Asthma, peripheral eosinophilia, peripheral neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities remain the major classification criteria of the disease [1].

Our patient had been diagnosed with bronchial asthma over 25 years before admission. Skin lesions are the most common extra-pulmonary findings in the course of the disease. The main types of skin lesions reported include erythematous maculopapules resembling erythema multiform, hemorrhagic lesions ranging from petechiae to extensive ecchymosis, often associated with wheals, necrosis and ulceration, and cutaneous and subcutaneous nodules that are usually deep-seated and tender with a predilection for the scalp and temple region. Others report livedo reticularis, papulovesicles, non-pitting periorbital edema, and nail-fold infarcts [4-8]. Skin nodules urticarial rash and necrotic bullae all over the body were found in our patient. Regarding clinical features, our patient complained of progressive dyspnea, productive cough, pain of elbows and knees, and 40°C fever, despite the antibiotic and steroid treatment. We also found radio-logic changes, such as pulmonary infiltrates detected by X-ray and computed tomography, and other manifestations including, mononeuritis multiplex, eosino philic endomyocarditis, with no renal involvement documented. These are consistent with main clinical findings describing the disease [1,3-5]. We did not find positive antineutrophil cytoplasmic antibodies (ANCA) in our patient. According to Reid et al [9] and Della Rosa et al [10] positive ANCA may be found in 39 to 59% of patients with CSS. Sable-Fourtassou et al [11] found the predominance of glomerulonephritis and peripheral neuropathy among positive ANCA patients, and more frequent cardiac involvement, and fever, among ANCA negative patients. Based on that, these authors hypothesized that ANCA positivity may reflect an active vasculitic process, while ANCA negativity may be more frequently linked to another clinical phenotype that could correspond to different pathogenic mechanisms which are less often associated with ANCA. Consistently with their hypothesis, cardiac involvement and no gromerulonphritis characterized our case, with the exception for peripheral neuropathy presented. According to Sable-Fourtassou et al [11] a higher frequency of vasculitis in biopsy specimens from the ANCA-positive group and a close association of renal involvement with ANCA positivity support a pathogenic role for ANCA. Kallenberg et al [12] focusing on ANCA directed to proteinase-3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA) summarized that ANCA alone are not sufficient, as based on clinical and experimental data, and other, probably exogenous factors, seem necessary for disease induction and (re)activation. In particular, silica, the carriage of Staphylococcus aureus, and various genetic factors may underline susceptibility to the disease. We did not find any bacterial source of infection or any genetic factors in our patient. Concerning treatment, our patient received a conventional prednisone therapy, resulting in early regression of clinical features. Further dose of the drug was titrated according to the level of blood eosinophils, with no signs or symptoms of the disease during a follow-up of 18 months of therapy. Despite novel therapies being introduced, systemic corticosteroids are still considered the cornerstone treatment [13]. Swietlik and Doboszynska [14] suggest the use of inhaled corticosteroids as a recurrence preventing approach and this may be a further step to be taken in our patient. Lamprecht et al [15] in their most recent publication summarize that anti-CD20 therapy and TNF-alpha-antagonists may represent strategies for refractory disease, but evidence from controlled trials is still lacking [15].

Conflicts of interest

The authors declare that they have no competing interests.