Eosinophilic myositis in calpainopathy: could immunosuppression of the eosinophilic myositis alter the early natural course of the dystrophic disease?

An 11-year-old girl with a calpain-3 gene (CAPN-3) mutation and eosinophilic myositis on muscle biopsy had high serum CK levels and eosinophil counts which showed spontaneous fluctuations. After commencement of immunosuppressive therapy reciprocal changes occurred in response to alterations in doses of the medications. Subacutely evolving and spreading muscle weakness developed during tapering of the immunosuppressive medications. These observations suggest that either the occurrence of eosinophilic myositis or the withdrawal of the immunosuppressive treatment may have accelerated the clinical course of the calpainopathy in this case. The positive effect of immunosuppressive therapy might have implications for the management of calpainopathy with an inflammatory component.

Insuficiência respiratória aguda como manifestação da síndrome de eosinofilia-mialgia associada à ingestão de L-triptofano / Acute respiratory failure as a manifestation of eosinophilia-myalgia syndrome associated with L-tryptophan intake

A síndrome da eosinofilia-mialgia foi descrita em 1989 em pacientes que apresentavam mialgia progressiva e incapacitante e eosinofilia sérica, nos líquidos e secreções. A maioria dos pacientes relatava uso prévio de L-triptofano. Sintomas respiratórios são relatados em até 80 por cento dos casos, eventualmente como manifestação única. O tratamento inclui suspensão da droga e corticoterapia. Relatamos o caso de uma mulher de 61 anos com insuficiência respiratória aguda após uso de L-triptofano, hidroxitriptofano e outras drogas. A paciente apresentava eosinofilia no sangue, lavado broncoalveolar e derrame pleural. Após a suspensão da medicação e corticoterapia, houve melhora clínica e radiológica em poucos dias.

Eosinophilia-myalgia syndrome was described in 1989 in patients who presented progressive and incapacitating myalgia and eosinophilia in blood, fluids and secretions. Most patients report previous L-tryptophan intake. Respiratory manifestations are found in up to 80 percent of the cases, occasionally as the only manifestation. Treatment includes drug discontinuation and administration of corticosteroids. Here, we describe the case of a 61-year-old female admitted with acute respiratory failure after using L-tryptophan, hydroxytryptophan and other drugs. The patient presented eosinophilia, together with elevated eosinophil counts in the bronchoalveolar lavage and pleural effusion. After discontinuation of the drugs previously used, corticosteroids were administered, resulting in clinical and radiological improvement within just a few days.

[Acute respiratory failure as a manifestation of eosinophilia-myalgia syndrome associated with L-tryptophan intake]. / Insuficiência respiratória aguda como manifestação da síndrome de eosinofilia-mialgia associada à ingestão de L-triptofano.

Eosinophilia-myalgia syndrome was described in 1989 in patients who presented progressive and incapacitating myalgia and eosinophilia in blood, fluids and secretions. Most patients report previous L-tryptophan intake. Respiratory manifestations are found in up to 80% of the cases, occasionally as the only manifestation. Treatment includes drug discontinuation and administration of corticosteroids. Here, we describe the case of a 61-year-old female admitted with acute respiratory failure after using L-tryptophan, hydroxytryptophan and other drugs. The patient presented eosinophilia, together with elevated eosinophil counts in the bronchoalveolar lavage and pleural effusion. After discontinuation of the drugs previously used, corticosteroids were administered, resulting in clinical and radiological improvement within just a few days.

[Diagnostic problems in eosinophilic fasciitis]. / Problemy diagnostyczne zwiazane z eozynofilowym zapaleniem powiezi.

We presented two cases with symptoms of diffuse swelling of subcutaneous tissue, stiffness and tenderness of involved areas, fever, eosinophilia and hypergammaglobulinemia. The inflammatory infiltrates consisting of lymphocytes, plasma cells and eosinophils were yielded in fascia. The difficulties in differentition of the symptoms between eosinophilic fasciitis and "eosinophilia-myalgia syndrome" are discussed.

[Eosinophilic myositis in a 9 year old boy]. / Miositis eosinofílica en un niño de 9 años.

INTRODUCTION: Eosinophil infiltration of skeletal muscle is rare, but often no etiological factor can be identified and these are isolated eosinophilic myositis. They may be associated with parasite infections or drugs, or be features of rare systemic disorders of hypereosinophilia, such as the myalgia eosinophilia syndrome and the idiopathic hypereosinophilic syndrome. The eosinophilic myopathies should be distinguished from the commoner inflammatory myopathies such as polymyositis and dermatomyositis. CLINICAL CASE: A nine year old boy with slight motor clumsiness but normal psychomotor development and neurological findings. Laboratory findings showed slightly raised serum transaminases (SGOT 271, SGPT 157 UI/L), CPK 7517 UI/L and eosinophilia (707/mL). Investigations for myoglobin cysticercosis, trichinosis, hydatidosis and toxicariasis were negative. No parasites were found in the faeces. The gammaglobulins were normal. Anti smooth muscle, antinuclear and anti KLM antibodies were negative. Cardiological studies were normal. His father, mother and two siblings had normal results of laboratory tests. Muscle biopsy showed inflammatory myopathy with abundant eosinophils, no evidence of parasites, no alteration of membrane proteins: dystrophin, sarcoglycan and merosine. Two years later he remains asymptomatic, maintains raised muscle enzyme levels in all tests with figures for CPK between 3,065 and 9,616UI/L, and eosinophilia ranging between 634 and 1,026/mL. Corticosteroid treatment was tried but no response obtained. CONCLUSION: We consider this to be a case of eosinophilic polymyositis which gives rise to many questions regarding etiopathogenesis, management and prognosis.

Eosinophilia-myalgia syndrome and giant cell myocarditis: a case report and therapeutic approach.

Eosinophilia-myalgia syndrome and giant cell myocarditis are rare and unrelated inflammatory conditions. Both may result in intense inflammatory infiltrates with eosinophilic predominance. A case involving a patient in whom both conditions occurred and who required intensive, prolonged immunosuppressive therapy is presented.

[Neuromuscular diseases with eosinophilia]. / Les maladies neuromusculaires avec eosinophilie.

Neuromuscular diseases with eosinophilia include a number of disorders associated with variable degrees of muscle, peripheral nerve, and connective tissue involvement. Eosinophilic infiltration in blood and/or tissue is a consistent finding. In addition to the neurologic manifestations of systemic vascularitis, in particular Churg and Strauss syndrome, there are three main forms of neuromuscular disease. Diffuse fasciitis or Shulman syndrome which can be limited to the fascia or associated with perimyositis is sensitive to corticosteroids. Eosinophilic myositis corresponds to focal muscle involvement and is also sensitive to corticosteroids. Eosinophilic polymyositis is a manifestation of essential hypereosinophilic syndrome and is life-threatening. Eosinophilia-myalgia syndrome and toxic oil syndrome are separate entities that occur in outbreaks and involve poisoning by ingestion of L-tryptophan and adulterated oil containing aniline respectively. The key to diagnosis of these neuromuscular diseases is muscle biopsy to detect the presence of polynuclear eosinophils.

Elevated L-kynurenine level and its normalization by prednisolone in a patient with eosinophilia-myalgia syndrome.

We report a L-tryptophan-induced case of eosinophilia-myalgia syndrome in a Japanese woman and describe the time course of changes in tryptophan metabolism observed during steroid therapy. She had taken 1.0 g of the implicated L-tryptophan daily. When admitted due to painful swelling of her extremities, eosinophil count was 22.3 x 10(9)/L. Before prednisolone treatment, her serum L-kynurenine level was 10.2 mumol/L, a level about three-fold higher than the normal value, while serum tryptophan level was abnormally low (23.1 mumol/L). On the 14th day of prednisolone treatment (40 mg daily), L-kynurenine was declined to 8.1 mumol/L and, concomitantly, L-tryptophan level increased to the normal range (51.0 mumol/L). Subsequently, on the 42nd day of therapy, serum L-kynurenine was normalized. In contrast, serum serotonin level was unchanged throughout the course of this therapy. Prednisolone dramatically reduced the elevated serum L-kynurenine with a reciprocal increase in serum L-tryptophan indicates that abnormal tryptophan metabolism, may play a role in the pathogenesis of eosinophilia myalgia syndrome, and that the observed effect of steroid treatment was due to suppression of elevated activity of indoleamine 2, 3-dioxygenase, a first rate-limiting enzyme of the kynurenine pathway.

Eosinophilic fasciitis: report of a case diagnosed 14 years after its onset.

The authors describe a case of eosinophilic fasciitis diagnosed 14 years after the onset of the clinical and laboratory manifestations of the disease. Failure to carry out an adeguate histopathological examination during this period played a role in delaying diagnosis.

The eosinophilia-myalgia syndrome: status of 205 patients and results of treatment 2 years after onset.

OBJECTIVE: To describe the course of the eosinophilia-myalgia syndrome during a 2-year period. DESIGN: 15 physicians completed a structured review form to describe symptoms, physical findings, laboratory data, and responses to treatments in 205 patients with the eosinophilia-myalgia syndrome at the onset of illness and after 18 to 24 months of follow-up. SETTING: 15 university and private clinical practice settings. PATIENTS: 205 patients for whom follow-up data were available and who met four criteria at diagnosis: eosinophil count of 1000 cells/mm3 or greater; presence of fasciitis, peripheral neuropathy, polyradiculopathy, interstitial pulmonary disease, pulmonary hypertension, or myocardial involvement; history of L-tryptophan consumption; and absence of other conditions that could account for these findings. INTERVENTION: Empirical interventions by the physicians. MEASUREMENTS: Symptoms, physical findings, laboratory test results, biopsy findings, radiographic reports, therapeutic interventions, and responses to these interventions. RESULTS: After 18 to 24 months, all symptoms except cognitive changes were reported to have improved in most patients. Nearly all physical findings were also reported to have improved or resolved in most patients; only peripheral neuropathy was unchanged. No evidence of ongoing inflammatory disease was reported. Prednisone was reported to be helpful in 79% of patients who received it during the acute phase of the syndrome. No other treatment was reported to be consistently beneficial. CONCLUSIONS: 18 to 24 months after the onset of illness, most symptoms and physical findings in most patients with the eosinophilia-myalgia syndrome resolved or improved. Cognitive changes were reported to be worse in 32% of patients. Prednisone was helpful in the acute phase of illness. No treatment was clearly valuable in management of the later phase of the syndrome.

Head and neck manifestations of eosinophilia-myalgia syndrome.

Eosinophilia-myalgia syndrome (EMS) is a multisystemic disease that occurs in patients who have consumed products containing L-tryptophan. Prominent features include incapacitating myalgias, arthralgias, neuropathies, and eosinophilia. Despite the frequent association of dysphagia, dyspnea, and the potential for aspiration, the otolaryngology literature is devoid of information on EMS. In order to determine the frequency of otolaryngic symptoms, questionnaires were distributed to patients with EMS in 33 different US states. Among the 28 various head and neck manifestations studied, 70% of EMS patients complained of generalized muscle spasms, 66% xerostomia, 62% dyspnea, and 56% dysphagia. In addition, the epidemiology, clinical presentation, diagnostic criteria, and treatment options are discussed. This paper assesses the frequency of otolaryngic manifestations of EMS, as well as introduces this syndrome to the otolaryngologist-head and neck surgeon. It is important for the otolaryngologist to be aware of EMS and its manifestations and treatments so that patients with this potentially lethal disease can receive appropriate evaluation and expeditious treatment.

Eosinophilic rheumatic disorders.

Almost any rheumatic disorder can occasionally be characterized by the presence of eosinophilia, but there are only a few in which eosinophilia is a defining characteristic. These include eosinophilic fasciitis as well as toxin-induced disorders such as eosinophilia-myalgia syndrome and toxic oil syndrome. The epidemiology, clinical features, and pathogenesis of these conditions are reviewed in this article, and a rational approach to management of these entities is discussed.

Serum sickness secondary to ciprofloxacin use.

Although serum sickness-like reactions are uncommon, various drugs have recently been implicated to manifest the reaction. The following case report is of a possible serum sickness-like reaction secondary to ciprofloxacin use, a commonly prescribed antibiotic in the US. A 62-y-old female developed polyarthralgias, myalgia and a generalized urticarial rash following 5 d use of ciprofloxacin. On admission to the hospital, patient was placed on cefazolin and gentamicin for suspected bacteremia. However, the regimen was discontinued after 72 h because of worsening clinical condition. Patient was placed on iv methylprednisolone therapy, and within 18 h a significant improvement was noted in her myalgias and rash. Over the next 72 h the steroid therapy was changed to a po regimen and the patient became asymptomatic 5 d after the initiation of steroid therapy. Patient was discharged on day 9 of hospital admission. Though serum sickness-like reactions have been reported with various drugs, only 1 case has been reported implicating ciprofloxacin. Clinicians should be aware of this potential adverse event secondary to ciprofloxacin use.

Eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia.

Eosinophilia-myalgia syndrome reached epidemic proportions in 1989. Its precise etiology remains uncertain, yet virtually all cases were associated with the ingestion of L-tryptophan containing trace amounts of several chemicals. Clinical and pathologic features of eosinophilia-myalgia syndrome are similar to those of the toxic-oil syndrome, which occurred in Spain in 1981 in association with the ingestion of adulterated rapeseed oil. During the past year, the epidemiology of eosinophilia-myalgia syndrome has been better defined, with a second trace contaminant linked to this condition. Knowledge of the clinical and histopathologic features of eosinophilia-myalgia syndrome has also expanded. These and other important advances in the understanding of eosinophilia-myalgia syndrome, toxic-oil syndrome, and diffuse fasciitis with eosinophilia are presented.