Drug-related Churg-Strauss syndrome?

Churg-Strauss syndrome (CSS) is a rare disease belonging to the group of necrotizing vasculitides affecting medium and small vessels, classified together with Wegener granulomatosis and microscopic polyarteritis. The literature is reviewed concerning vasculitides associated with drug use, focusing on CSS. A representative case of CSS is reported, in whom the possibility could not be excluded that oestrogen replacement therapy contributed to the onset of CSS. The case of a 56-year-old female patient is presented who had a history of allergic rhinitis and steroid-dependent asthma for years. To prevent postmenopausal complaints and further loss of bone density, she received oestrogen replacement therapy. After three months of hormone therapy, signs of CSS appeared. Oestrogen administration (1 mg norethisterone acetate, 1 mg oestriol and 2 mg oestradiol daily) was stopped. The diagnosis was confirmed by the clinical appearance, laboratory tests and tissue biopsies. The patient received corticosteroids and cyclophosphamide treatment and subsequently the eosinophil count returned to normal within two weeks and her condition improved significantly.     

Liver metastases from breast cancer 14 years after radical mastectomy; successful treatment with hepatic arterial infusion chemotherapy and systemic endocrine therapy--a case report

The patient was a 73-year-old woman who was admitted to our hospital for epigastric discomfort and body weight loss. She had undergone radical right mastectomy in March 1982. Ultrasonography and computed tomography revealed multiple tumors in the liver. Three of the tumor markers (CA-125, CA 19-9, and CA 15-3) were positive. US-guided fine needle aspiration biopsy of the liver tumor showed small atypical cells with solid cell nests. Immunohistochemical tests revealed estrogen receptor was positive. We diagnosed the patient as recurrent breast cancer metastatic to liver, 14 years postoperatively. The liver tumors were successfully controlled with the combination of intra-arterial infusion chemotherapy (5-fluorouracil, epirubicin, and mitomycin C) along with systemic endocrine therapy (medroxyprogesterone acetate and fadrozole hydrochloride hydrate).     

Unilateral hypoglossal nerve paralysis following the use of the laryngeal mask airway

We report a unilateral hypoglossal nerve paralysis following the use of a laryngeal mask airway in a 62-year-old woman with rheumatoid arthritis undergoing a shoulder joint replacement. Cervical epidural anaesthesia was combined with general anaesthesia using nitrous oxide administered via a laryngeal mask airway with the patient in the right lateral decubitus position. The next morning, the patient was noted to have a right hypoglossal nerve palsy. Compression of the nerve between the laryngeal mask airway cuff, distended with nitrous oxide, and the hyoid bone, was considered to be the cause of the nerve paralysis.     

Cricothyroid joint injury

We report a rare case of Churg-Strauss syndrome (CSS) in a 41-year-old Japanese man with a history of middle-age onset of bronchial asthma who had severe abdominal pain. He presented with ileus caused by an annular ulcer of the ileum, attributable to mucosal ischemia resulting from necrotizing vasculitis of the mesenteric artery. He also had marked hypereosinophilia (51.5%), elevated serum IgE levels (34040 IU/ml), and generalized enlargement of the superficial cervical lymph nodes, containing eosinophilic granulomas. A stenotic lesion caused by an annular ulcer in the ileum was found and resected by laparotomy. Microscopic examination of the resected specimen revealed luminal narrowing or occlusion of small arteries in the ulcer base, subserosa, and mesenterium resulting from marked fibrotic intimal thickening with fragmentation or lack of the internal elastic lamina. These findings were diagnosed as vasculitis, scar stage. The postoperative course was uneventful, with the patient receiving a maintenance dose of prednisolone (10-15 mg/day) for 7 years subsequently. We must carefully diagnose and treat patients with middle-age onset asthma, because the symptom may be a lung manifestation of CSS, in which various organs including gastrointestinal tract are involved as a result of systemic necrotizing vasculitis.     

Churg-Strauss syndrome. Personal caseload and review of the literature

Churg-Strauss syndrome (CSS) is a disease characterised by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. In the course of CSS three phases may be distinguished. The prodromal phase, which may persist for many years, consists of allergic disease. The second phase is characterised by peripheral blood eosinophilia and eosinophilic tissue infiltrates that produce a clinical picture diagnosed as Loeffler's syndrome, chronic eosinophilic pneumonia or eosinophilic gastroenteritis. The third phase is dominated by systemic vasculitis in which skin, cardiovascular system, gastrointestinal tract and peripheral nervous system are frequently involved. Renal disease in CSS is less common and generally less severe than that classical polyarteritis nodosa and Wegener's granulomatosis. Genitourinary tract may be involved, too. In the postvaculitic phase, allergic rhinitis and asthma usually persist and clinical picture is characterised by the consequences of the vasculitic illness, most commonly in form of neuropathy and hypertension. The pathogenesis of CSS is unknown but its association with asthma and allergic rhinitis may indicate an abnormal immune reactivity. The recently reported association with antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity may suggest a their role in the pathogenesis of the disease. An important role may be played by eosinophils, too. The main therapy is that with corticosteroids, possibly in association with immunosuppressive drugs.     

Intermittent chyluria in a young man

Chyluria is the passage of chylus into urine resulting in fistulization through the lymphatic system and the urinary system. This rare condition is usually caused by filaria infestation or malformations, neoplasia or trauma. We report a case of a 18-year-old man. The patient presented milky urine which had appeared after angiography following minor leg trauma. Physical examination revealed asymmetry of the face and cutaneous dyschromia. Blood tests revealed hypogammaglobulinemia and altered CD4/CD8 ratio (0.6). Urine tests showed proteinuria (30 mg/dl), lipiduria (triglycerides 750 mg/dl) and density of 1025. Renal function was normal. Abdomen computed tomography and urography were normal. Cystoscopy revealed the presence of milky urine in the bladder and selective catheterization revealed that the origin was the right ureter alone. Ascendent pyelography did not reveal any malformation of the urinary tract; but after this the chyluria spontaneously disappeared. The patient was rehospitalized 3 months later for recurrence. Lymphography was then performed and revealed a dilated lymphatic network with minute lacunar images projecting into the right kidney. Chyluria again disappeared spontaneously and recurred sporadically over the next two years in a patient who remained in good physical condition. The etiology of chyluria in a patient without filaria infestation is problematic, particularly when the most common causes (tuberculosis, neoplasia, trauma) are excluded as in our case. The asymmetry of the face, together with cutaneous dyschromia and the presence of a subarachnoidea cyst in the right temporal region suggested our patient had multiple congenital malformations.     

Clinical reasoning and decision making in the practice. A woman with abdominal complaints

A female patient aged 28 years suffered from recurrent episodes of diarrhoea. Giardia lamblia was isolated once. By rigorous application of basic clinical skills, such as thorough history taking and physical examination, together with laboratory tests the correct diagnosis was established (in this patient systemic lupus erythematosus). Two important aspects of clinical reasoning, viz. returning to the patient when new information becomes available (cyclic way of working) and pattern recognition ensured a fruitful diagnostic process.     

Laryngeal Kaposi's sarcoma in patients with AIDS

Over a period of 10 years 17 human immunodeficiency virus(HIV)-infected patients with laryngeal Kaposi's sarcoma were seen and treated at University College London Hospitals. All patients had advanced HIV disease. Their presentation was with symptoms of upper airway obstruction in the majority of cases and the diagnosis was made by fibreoptic examination of the larynx. Biopsy was associated with brisk haemorrhage in one patient, who required a temporary tracheostomy, and was not performed in the other 16 cases. The commonest site of laryngeal involvement was the supraglottis in 11 patients, with glottic lesions noted in eight patients: subglottic lesions were seen in only three. Treatment of laryngeal Kaposi's sarcoma was, in general, conservative, five patients received low dose radiotherapy to the larynx and 10 were treated with systemic chemotherapy for disseminated Kaposi's sarcoma. Laryngeal Kaposi's sarcoma did not contribute to patient mortality.     

Response to treatment with progesterone in a patient with pulmonary lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is defined as an abnormal proliferation of smooth muscles around lymphatics, venules, and brochioles. This article describes our experiences treating a 21-year-old, white female who experienced recurrent shortness of breath during air travel last year. Her episode was severe and the patient was transported to the hospital as soon as the airplane landed. Physical exam in the emergency room was significant for absent breath sounds on the right side and the chest X-ray revealed a pneumothorax. She required two chest tubes for complete lung re-expansion. Further evaluation showed an obstructive pattern and air trapping on pulmonary function tests. This patient was treated with Medroxyprogesterone acetate (MPA) for six months and subsequent pulmonary function tests revealed improvement in her condition.     

Pregnancy in granulomatous vasculitis

OBJECTIVE: To study the fetal and maternal outcome of pregnancy in patients with granulomatous vasculitis. METHODS: Four pregnancies in two patients with Wegener's granulomatosis (WG) and one patient with Churg-Strauss syndrome (CSS) were identified and followed in our specialised clinic for pregnancy and connective tissue diseases. RESULTS: Three pregnancies ended with live babies and one with intrauterine death at 25 weeks of gestation. One WG patient remained in remission throughout pregnancy and the other experienced severe activity at 12 weeks. The CSS patient was in remission during her first pregnancy, but the disease flared severely in the second. CONCLUSIONS: Pregnancy in patients with granulomatous vasculitis requires preconceptual planning, careful clinical management, and vigorous treatment of active disease.     

Amebic liver abscess of unusual presentation

A 35-year-old male who had travelled extensively in the tropics presented with severe anorexia and vomiting associated with fever of 39-40 degrees C during a 4-day period. The clinical findings were entirely negative. In 1973, he had been given metronidazole for amebic dysentery, since when recurrent attacks of diarrhea and abdominal pain had been treated with iodoquinoleines. Stool examination was negative for amebae. Liver scan revealed a suspect "expansive process" in the right lobe. The presumptive diagnosis of amebic abscess was made and metronidazole therapy was started. In less than 24 h the patient became afebrile. The abscess was confirmed by a further liver scan. The definitive diagnosis of amebiasis was established 16 days later when the immunofluorescence level, which had been previously negative, became positive 1/480. This case demonstrates the dangers of the indiscriminate use of iodoquinoleines in patients who have travelled in tropical countries. The amebic liver abscess may be silent locally while causing systemic manifestations such as fever. Early treatment of hepatic amebiasis is recommended even with a presumptive diagnosis. Serological tests during the development of an amebic abscess may be negative and should be repeated after several days of therapy.     

Tuberculosis of the larynx

Laryngeal tuberculosis was diagnosed in two men, a 73-year-old man Dutch by birth and a 40-year-old one Turkish by birth. In the former patient it was probably primary tuberculosis, in the latter secondary (he had lung tuberculosis as well). The clinical picture was highly suggestive of laryngeal carcinoma in both patients. They both recovered with chemotherapy. Laryngeal tuberculosis may mimic laryngeal carcinoma. The diagnosis is based on Ziehl-Neelsen staining, culture and polymerase chain reaction (PCR) on Mycobacterium tuberculosis. Because laryngeal tuberculosis is highly infectious, the patient has to be nursed in isolation and people in his or her environment have to be screened. The response of laryngeal tuberculosis to chemotherapy is good.     

Parathyroid carcinoma: diagnosis and management

A patient with severe hypercalcemia and a palpable neck mass is presented. The highest calcium was 18.8 mg/dL. A left lower neck mass was felt on examination. The trachea was deviated to the right side on a chest film. A barium swallow demonstrated an indentation on the left side of the esophagus. An en-bloc resection of the mass including the thyroid lobe, the strap muscles, and the recurrent laryngeal nerve was done. The pathologic specimen revealed parathyroid carcinoma with dense fibrous septae, invasion of the capsule, and vascular invasion. The patient is alive and without evidence of hypercalcemia or recurrence of the disease 23 years after surgery, probably the longest survivor with carcinoma of the parathyroid gland. Parathyroid carcinoma should be suspected in any patient with severe hypercalcemia and a palpable mass. The best chance for cure is obtained by performing a wide surgical excision during the initial operation.     

A case of Paragonimus Miyazaki with pleuritis and meningoencephalitis

A 35-year-old man was admitted to our hospital with fever and headache. Chest X-ray revealed right pleural effusion. Lab tests revealed increase of eosinophils in his serum and pleural effusion. After admission he complained of doplopia and neck stiffness. Lumber puncture revealed eosinophilia in the cerbrospinal fluid. Brain CT and MRI showed characteristic images of meningoencephalitis. The patient had eaten raw Potamon dehaani and the case was diagnosed as paragonimus miyazaki after administration of intradermal reaction and Ouchterony's double diffusion test. The patient was successfully treated with praziqantel. It revealed that the pleural effusion and brain edema disappeared chest X-ray and brain MRI. This case can be considered as a characteristic example of Paragonimus Miyazaki with pleuritis and meningoencephalitis.     

Churg-Strauss syndrome with right ventricular tumor

HISTORY AND CLINICAL FINDINGS: A 47-year-old woman was admitted because of diarrhoea (3-5 stools daily) for 9 days, weakness for 3 weeks and painful wrist swelling, arthralgia and hyperesthesia over the medial aspect of the right thigh for 6 weeks. For 6 years she had been treated for asthma. Two operation had been performed for chronic sinusitis. ADMISSION FINDINGS AND INVESTIGATIONS: There was marked eosinophilia (5889/microliter) and thrombocytopenia (96,000/microliter), markedly increased serological inflammatory parameters and a raised total IgE level (134 IU/ml). The chest radiogram showed pulmonary infiltrates and bone marrow biopsy indicated eosinophilia. Echocardiography revealed a mass, 3 x 8 cm, in the ventral wall of the right ventricle with extension into the outflow tract. DIAGNOSIS, TREATMENT AND COURSE: These findings met the criteria of the American College of Rheumatology for Churg-Strauss syndrome (CSS). On methylprednisolone (1 mg/kg daily) the eosinophil and platelet counts became normal within 5 days and erythrocyte sedimentation rate and the level of C-reactive protein fell. Under cardiopulmonary bypass the tumour was removed, the tricuspid valve replaced, and the right ventricle reconstructed. Histology of the mass revealed it to be an organized thrombus. CONCLUSION: The relationship between the thrombus formation and CSS is unclear. Hypercoagulability connected with the inflammatory process may have played a part in the pathogenesis.     

Closest speaking space during the production of sibilant sounds and its value in establishing the vertical dimension of occlusion

The purpose of this investigation was to determine whether the production of sibilant sounds involved adopting a jaw position that corresponded to the closest vertical speaking space (CSS), by analysis of the smallest vertical excursion of the mandible during the performance of different phonetic exercises. A further objective was to establish the variability in the CSS produced by individual sibilant phonemes. Thirty young adult subjects had their CSS determined during three separate phonetic tests, using a kinesiograph (Sirognathograph, Siemens A.G., Benshiem, Germany) and a Bio-Pak (BioResearch Associates Inc., Milwaukee, WI) jaw-tracking software program. The first test was a general phonetic articulation test containing all the sounds of the English language and specifically including all six sibilant word sounds. The second phonetic test contained the six sibilant sound making up a short sentence. The third test included six single words, each expressing a different sibilant sound. No statistically significant difference among the mean CSS determined in each of three exercises was demonstrable. A phonetic test containing all sibilant sounds produced a CSS equivalent to that of a test containing all speech sounds. The vertical component of the CSS was also independent of the form or duration of the phonetic tests containing the sibilant word sounds used in this investigation. The CSS determined for 5 of the individual sibilant phonemes in the third exercise differed (p < 0.05) from that calculated for the three complete exercises. It was concluded that voicing sibilant phonemes, or word sounds, does cause the subject to adopt the CSS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Necrotizing myocardial vasculitis in Churg-Strauss syndrome: clinicohistologic evaluation of steroids and immunosuppressive therapy

Treatment of cardiac dysfunction associated with Churg-Strauss syndrome (CSS) is empiric since the histologic findings provided by endomyocardial biopsy are rare and often nondiagnostic. Myocardial necrotizing vasculitis presenting as restrictive cardiomyopathy has not been reported before. A case of CSS, presenting with fever and progressive heart failure due to pericarditis, eosinophilic endomyocarditis, and myocardial necrotizing vasculitis, is reported. Cardiac involvement assessed by noninvasive (cardiac two-dimensional echocardiogram and nuclear magnetic resonance [NMR] imaging) and invasive (cardiac catheterization, angiography, and biopsy) studies showed a moderate degree of pericardial effusion and left ventricular (LV) dysfunction (ejection fraction 0.40), severe diastolic dysfunction (increased right and LV filling pressure with a dip and plateau pattern) and a severe reduction of cardiac index (1.6 L/min/m2). Histologic characteristics showed marked eosinophilic infiltration of the endocardium and myocardium with myocitolysis and fibrinoid necrosis of arterioles, venules, and capillaries. Combination therapy of steroids and cyclophosphamide resulted in both a clinical (regression of pericardial effusion, normalization of systolic and diastolic dysfunction, and increase of cardiac index to 2.8 L/min/m2) and histologic (sequential endomyocardial biopsies at 1, 3, and 6 months of follow-up) resolution of cardiac involvement. No recurrences were registered at 12-month follow-up with the patient receiving a maintenance drug regimen.     

Thyroglossal duct cyst causing airway obstruction in an adult

Thyroglossal duct cysts, though not uncommon, rarely present with evidence of laryngeal compromise. The case presented is one of the very few cases with documented laryngeal invasion reported in the English language. Of clinical significance is the patient's presentation with laryngeal symptoms of choking and dysphonia in the presence of a small anterior cervical mass. While the thyroglossal duct cyst usually presents as an asymptomatic anterior neck mass, this case illustrates the importance of considering a thyroglossal duct cyst in any patient with airway compromise in the absence of a neck mass.     

Primary cutaneous cryptococcosis following trauma of the right forearm

A 62-year-old woman with sarcoidosis II, status post systemic steroid treatment, developed an inflammatory, infiltrative skin lesion in the area of a traumatic haematoma of the right forearm. The clinical appearance at first corresponded to bullous erysipelas. Antibiotic therapy, which was instituted immediately, proved to be ineffective. A microbiological swab revealed infection with Cryptococcus neoformans. A systemic cryptococcosis could be excluded. Therapy with 200 mg itraconazole twice daily resulted in a prompt improvement.     

Respiratory insufficiency and absent left radial pulse after hemicolectomy

A 65 year old female developed right thoracic pain, productive cough and fever four weeks after hemicolectomy because of a cancer of the sigmoid. In spite of antibiotic treatment the condition of the patient deteriorated and she was admitted to the hospital with pneumonia of an upper lobe. Chest X-ray visualized prominent proximal pulmonary arteries. Progressive respiratory failure developed and blood gas analysis revealed hypocapnic hypoxemia. The patient had to be intubated and ventilated mechanically. Later, left arm blood pressure measurements could no longer be taken and the radial pulse was missing. Thereafter, an ischemic syndrome of the right leg developed. Embolectomy from the superficial femoral artery was carried out the same day. The patient died five days later. Autopsy revealed an almost complete occlusion of the pulmonary arteries. The organization of thrombotic material indicated recurrence. Emboli were also found in the systemic circulation. A large patent foramen ovale together with signs of pulmonary arterial hypertension are indicative of paradoxical thromboembolism.