[Polymyositis/dermatomyositis--clinical picture and treatment]. / Polimiozitis/dermatomiozitis--klinicka slika i terapija.

The clinical presentation ofmyositis ranges from a painless muscle weakness to significant myalgia with muscle weakness and constitutional symptoms. Along with muscle and skin affection and constitutional symptoms, the disease can affect lungs, joints, heart and gastrointestinal system. It is important to note that the clinical presentation ofmyositis syndrome may overlap with symptoms of other connective tissue disease in overlap syndromes (SLE, SSCL, RA, SSjö). Common manifestations of the disease are weakness and muscle fatigue, which is the result of skeletal muscles inflammation (usually the proximal group of muscles, bilaterally and symmetrical). Severe forms of the disease with affection of the throat and respiratory muscles can vitally endan- ger patients. Among constitutional (general) symptoms, fever, malaise and weight loss are usually expressed. Skin affection in dermatomyositis can be localized or generalized like vesiculobullous erythroderma. Pathognomonic cutaneous manifestations of dermatomyositis are Gottron's papules and heliotrope erythema. Lungs are most commonly affected organs (with exception of muscles and skin) in polymyositis and dermatomyositis. The affection of lung can sometimes result in fatal outcome (interstitial lung disease, secondary pulmonary hypertension). Cardiac affection is usually subclinical, but can also be expressed as heart failure, acute coronary syndrome or conduction disturbances. Infrequent manifestations of the disease are gastroesophageal reflux, malabsorption, gastrointestinal mucosal ulceration, soft tissue calcification, Raynaud's syndrome, arthralgia/arthritis and some other less common clinical manifestations of the disease. Treatment of polymyositis/dermatomyositis includes immunosuppressive/immunomodulatory therapy and supportive, symptomatic treatment. The basis for myositis treatment are glucocorticoids, which are applied orally in a daily dosage regimen of 0.75 to 1 mg/kg/day, and in severe forms of the disease in the i.v. pulse doses of 1 g/day. Immunosuppressants/immunomodulators are added in the therapy along with glucocorticoids for better control of the disease and to reduce the required dose of glucocorticoids (side effects of longterm high doses glucocorticoide use). The most commonly used immunosuppressive drug is methotrexate at a dose of up to 25 mg/week. Hydroxychloroquine has a good effect on the cutaneous manifestations of the disease. Among other immunosuppressants which are used in the treatment of myositis are azathioprine, cyclosporine (in patients with pulmonary affection), mycophenolate mofetil and tacrolimus. Intravenous immunoglobulins applied parenterally in a dose of 2 g/kg divided into multiple doses showed an excellent clinical effect in patients with affection of the esophagus and throat muscles, in patients with pulmonary affection and in patients with resistant disease. The experience with the biologics is limited to a small number of patients. Physiotherapy is a necessary form of treatment for the recovery of muscle strength in the remission phase of the disease. A prompt treatment of infections and heart failure is sometimes life-saving in patients with myositis. Symptomatic treatment of pain with analgesics and NSAIDs reduces pain, speeds up recovery and improves the quality of life in patients with myositis.

Therapeutic effect of hyperbaric oxygen on inclusion body myositis.

An inflammatory myopathy, inclusion body myositis (IBM) presents with progressive muscle weakness against a background of elevated creatine kinase and diffuse endomysial damage. Typically occurring in patients greater than 50 years of age, it is commonly misdiagnosed as polymyositis or other rheumatological disease and is often ineffectively treated with steroids [1]. The approach to IBM is frequently a clinical challenge due to its unique and often aberrant response to common treatment modalities. Here we report an apparent improvement in the clinical course of and associated laboratory findings in a patient with co-existing IBM following the use of hyperbaric oxygen therapy as an adjunct for managing ischemic colitis.

Rare causes of calcitriol-mediated hypercalcemia: a case report and literature review.

CONTEXT: Calcitriol-mediated hypercalcemia resulting from elevated extrarenal 25-hydroxyvitamin D-1alpha-hydroxylase (1alpha-hydroxylase) activity has not previously been described in giant cell polymyositis. CASE: We report an unusual case of hypercalcemia due to disseminated granulomatous disease in a 62-yr-old woman with profound proximal muscle weakness and weight loss. She was initially diagnosed with vitamin D deficiency myopathy with a low serum 25-hydroxyvitamin D; serum calcium at this time was low-normal. Vitamin D(3) 3000 IU daily was prescribed. One month later, blood work showed new hypercalcemia and hypercalciuria with normalized 25-hydroxyvitamin D. 1,25-dihydroxyvitamin D was high-normal, despite a suppressed PTH, undetectable PTHrP, and essentially normal renal function. Her hypercalcemia resolved, and her strength improved only after prednisone was added to bisphosphonate therapy. Two weeks later, she died from acute congestive heart failure. METHODS AND RESULTS: Autopsy revealed a disseminated giant cell myositis affecting skeletal, cardiac, and gastrointestinal smooth muscle. Immunohistochemistry localized 1alpha-hydroxylase to the inflammatory infiltrates in skeletal and cardiac muscle. EVIDENCE: A review of English publications in Medline and Embase, including a reference search of retrieved articles, revealed that calcitriol-mediated hypercalcemia has been described in over 30 conditions, most of which are granulomatous in nature, ranging from inflammatory conditions and foreign body exposures to infections and neoplasms. CONCLUSIONS: Hypercalcemia resulting from autonomous 1alpha-hydroxylase activity may be unmasked by low-dose vitamin D supplementation and should not be excluded from the differential diagnosis of nonparathyroid causes if the serum calcitriol is inappropriately normal, rather than frankly elevated.

Carcinoma tubárico primitivo y polimiositis / Primary primitive fallopian tube carcinoma and polymiositis

Los autores aportan un caso de asociación de un carcinoma tubárico primitivo a una polimiositis. Se trata de una paciente de 53 años, que consultó por dolores pelvianos, metrorragias y leucorreas amarillentas, con una masa pelviana de difícil movilización y móvil a la exploración clínica, y una masa laterouterina derecha con hidrosálpinx en la ecografía pelviana. El diagnóstico definitivo lo aportó el examen ana tomoclínico de la pieza de anexectomía derecha. La histerectomía total sin conservación anexial no dejó residuo tumoral. La paciente perdió contacto con nosotros sin tratamiento complementario y reconsultó 3 años después por una impotencia funcional de los miembros superiores, seguida de los inferiores, mialgias, alteraciones de la deglución y aparición de una adenopatía supraclavicular izquierda. Los exámenes clínicos, paraclínicos y anatomopatológicos mostraron que se trataba de un adenocarcinoma tubárico derecho con recaída ganglionar pelviana y metastásica supraclavicular izquierda, con polimiositis paraneoplásica. La paciente se ha beneficiado de 6 sesiones de quimioterapia, después de radioterapia en el hueco subclavicular izquierdo, a la dosis de 45 Gy. La respuesta al tratamiento ha sido excelente, y la paciente está en remisión completa después de un margen de 50 meses tras el diagnóstico. La asociación de cáncer de la trompa con una polimiositis es excepcional, y es necesaria una terapia antineoplásica rápida para tratar el cáncer y la polimiositis paraneoplásica (AU)

We report a case of a primitive fallopian tube carcinoma associated with polymyositis. A 53­year-old woman consulted for pelvic pain, metrorrhagia, and leukorrhea. Physical examination revealed a renitent and mobile mass in the pelvis. Ultrasonography showed a right lateral uterine mass with hydrosalpinx. Pathological examination of the right annexectomy specimen provided the definitive diagnosis. No residual tumor was found at total hysterectomy with bilateral annexectomy. The patient was lost to follow-up for 3 years without complementary treatment and then consulted again for functional disability first of the upper limbs and subsequently of the lower limbs with myalgia, swallowing disorders and left supraclavicular node enlargement resulting from pelvic relapse of the right fallopian tube adenocarcinoma and left supraclavicular metastasis with paraneoplastic polymyositis. The patient was given 6 courses of chemotherapy with radiotherapy (45 Gy) centered on the left clavicular region. The patient showed excellent response to treatment, and remains in complete remission 50 months after diagnosis. The association of fallopian tube carcinoma with polymyositis is exceptional, requiring rapid treatment effective against the cancer and paraneoplastic polymyositis (AU)

[Dermatomyositis and polymyositis: clinical aspects and treatment]. / Dermatomyosite et polymyosite: aspects cliniques et traitement.

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.

[D-penicillamine-induced pemphigus, polymyositis and myasthenia]. / Pemphigus, polymyosite et myasthénie induits par la D-pénicillamine.

BACKGROUND: D-penicillamine can induce autoimmune disease, particularly in patients with associated immune disorders. CASE REPORT: A 67-year old woman who had been taking D-penicillamine for 15 months for rheumatoid arthritis was hospitalized due to the development of a bullous eruption and proximal muscle deficiency. Search for intercellular antisubstance antibodies in serum was negative. The skin biopsy histology revealed intra-epidermal cleavage in the mucosal body and direct immunofluorescence revealed epidermal frame-marking with anti-IgG and anti-C3 antibodies. Other tests revealed muscular cytolysis, and anti-acetylcholine receptor antibodies. The electromyogram showed neuromuscular block without muscle deficiency and muscle biopsy showed moderate myositis. D-penicillamine was interrupted and was followed by cure of the pemphigus and aggravation of the myositis, requiring high-dose systemic corticosteroid therapy. DISCUSSION: This patient developed D-penicillamine induced pemphigus, a rather frequent observation. The desmoglein immunolabelling favored drug-induced pemphigus and the course was rapidly favorable after withdrawal. Pemphigus had developed simultaneously with signs of myasthenia and polymyositis. Polymyositis and myasthenia are also known complications of D-penicillamine therapy. The association of these three complications suggests that D-penicillamine can unmask certain antigens or have an immunomodulator effect.