The Brazilian Society of Rheumatology recommendations on investigation and diagnosis of systemic autoimmune myopathies.

BACKGROUND: This research is recommended by the Myopathy Committee of the Brazilian Society of Rheumatology for the investigation and diagnosis of systemic autoimmune myopathies. BODY: A systematic literature review was performed in the Embase, Medline (PubMed) and Cochrane databases, including studies published until October 2018. PRISMA was used for the review, and the articles were evaluated, based on the Oxford levels of evidence. Ten recommendations were developed addressing different aspects of systemic autoimmune myopathy investigation and diagnosis. CONCLUSIONS: The European League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification stands out for the diagnosis of systemic autoimmune myopathies. Muscular biopsy is essential, aided by muscular magnetic resonance images and electroneuromyography in complementary research. Analysis of the factors related to prognosis with the evaluation of extramuscular manifestations, and comorbidities and intense investigation regarding differential diagnoses are mandatory.

Dermatitis artefacta mimicking cutaneous vasculitis: case report and literature overview.

A 31-year-old Caucasian woman with a history of anxiety disorder presented with chronic ulcerative lesions of the skin in arms and legs, treated initially as cutaneous vasculitis, evolving with relapsing during corticosteroid tapering, was diagnosed, after thorough investigation and no organic disease found, with dermatitis artefacta - a self-inflicted harm due to psychological disorders. Dermatitis artefacta is a rare condition, more frequent in women, in which traumatic skin lesions are caused by the patient him/herself, over accessible parts of the body, due to personality disorders. Clinicians should be aware of this alternative aetiology, especially as a differential diagnosis for refractory cutaneous vasculitis.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies.

BACKGROUND: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). MAIN BODY: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. CONCLUSIONS: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strength-building and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Aortic aneurysm in a patient with syphilis-related spinal pain and paraplegia.

The tertiary stage of syphilis is nowadays extremely rare, showing predilection for the cardiovascular and nervous systems. A 57-year-old Caucasian man sought medical assistance due to back pain that evolved to paraplegia of the lower limbs. A thoracic CT scan demonstrated an important aneurysmatic lesion of the descending thoracic aorta causing erosion of the vertebral bodies and VDRL and FTA-abs positivity. Although rare, syphilitic aortitis, the hallmark of cardiovascular syphilis, should be considered in the differential diagnosis in patients with thoracic aneurysm when in the absence of classic risk factors for atherosclerosis, especially in cases that progress with erosion of vertebral bodies.

Ainhum (dactylolysis spontanea): a case with hands and feet involvement.

Dactylolysis spontanea is a rare disease of unknown etiology, basically restricted to digits without systemic involvement. It evolves, in the form of a local constriction, affecting typically the distal phalanx of the fifth toe, rarely involving other toes and even more rarely involving fingers as in the case described herein.

Pericardial breast cancer metastasis 25 years after mastectomy.

Pericardial effusion in a patient with a history of cancer should always prompt a hypothesis of malignant involvement. We report the case of a 66-year-old white woman presenting with pericardial effusion 25 years after a mastectomy for ductal breast carcinoma. This is one of the cases with the latest recurrence ever reported.

Pulmonary metastases in men: primary tumor in an unusual location.

We report a case of breast cancer identified in a 72-year-old male as an accidental finding during the course of the investigation of a primary tumor and the search for pulmonary metastases. We address aspects related to the epidemiology, diagnosis, treatment and prognosis of this condition in males.