BACKGROUNDPulmonary arterial hypertension (PAH) is a rare and severe complication of SLE, with a worse prognosis and poor survival with estimated prevalence 0.5-17.5%. Divided in two cluster: vasculitic when acute associated to other systemic manifestations or vasculopathic when chronic and in isolated form. Lupus myocarditis is also a rare and severe manifestation. Both require rapid clinical suspicion, diagnosis and aggressive immunosuppressive treatment. CASE REPORTA 29-year-old female was initially evaluated in the emergency department describing oligoarthralgia of small joints, fatigue, loss weight of 26 kg in 5 months. Worsening of the symptoms in the last 4 days with facial swelling, malar rash and erythematous plaques on face and neck, oral ulcers and daily fever. At physical exam: recurrent bradycardia, but normal vital signs. The patient had previous diagnose of fibrous dysplasia in facial bones, and facial computed tomography (CT) scan demonstrated periorbital cellulitis and broad-spectrum antibiotics were initiated. Cardiomegaly in chest X-ray. Transthoracic echocardiogram with pericardiac effusion, moderated mitral insufficiency, mid-apical dyskinesia, right ventricular failure (30%), preserved left ventricular function. Estimated pulmonary arterial systolic of 43 mmHg. Chest CT angiography excluded pulmonary embolism and interstitial lung disease. In the meantime, immune panel was compatible is SLE with ANA 1/640 quasi-homogeneous pattern, anti-DNA antibodies positive (38.4), anti-RNP and IgM anticardiolipin in high titles, low c3 and c4 complement levels-and also elevated natriuretic peptide (1,090 pg/mL). Initiated intravenous methylprednisolone 1 g for 3 consecutive days and cyclophosphamide mensal pulse (0.5 g/m²/month) by 6 months followed prednisone 1 mg/kg/day (tapering 10 mg/month) plus hydroxychloroquine 5 mg/kg/day. CONCLUSIONAlthough the patient didn't present dyspnea and edema, fever, weight loss, bradycardia and cardiomegaly, the X-ray led us to clinical suspicion confirmed by echocardiogram. We describe a rare and severe case of vasculitic cluster of SLE-PAH associated to myocarditis and severe right ventricular failure. Rapid recognition and treatment were important for therapeutic success.

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Os coronavírus são importantes patógenos que acometem humanos e animais. Um novo subtipo de coronavírus foi identificado em 2019 como causador de casos de pneumonia, com rápida evolução para pandemia, com número crescente de infectados. A compreensão acerca da doença, sua prevenção e suas consequências durante e pós infecção estão em constante evolução e atualização. O presente artigo consta de revisão de periódicos internacionais acerta das manifestações reumatológicas no contexto e pós doença, condição essa que necessita de rápida avaliação diagnostica para melhorar o desfecho.

BACKGROUNDSystemic sclerosis is a connective tissue disease characterized by fibrosis and vascular and immunological abnormalities. The diffuse form is associated with high mortality. Progressive skin thickening, usually painful and pruritic, is the symptom that has the greatest effect on the patient's quality of life. The skin is a visible and accessible window to evaluate disease progression. In patients with the early form, skin involvement often begins distally, affecting the fingers, which become painful and swollen. This early edematous phase is sometimes misdiagnosed as inflammatory arthritis; however, after a few weeks, skin thickening appears. CASE REPORTFemale, 24 years old, white, previously healthy, had infection by the coronavirus, with mild symptoms, after receiving three doses of the vaccine. After eight weeks, she developed periorbital and lower limb edema, associated with dyspnea on minimal exertion, with complaints of paroxysmal nocturnal dyspnea and orthopnea. On admission physical examination, she was in a regular general condition, with tachypnea (24 incursions/min) and desaturation (87%), requiring the use of a nonrebreathing mask. On pulmonary auscultation, crackles. Laboratory tests with elevation of ALT (94 U/L), CRP (4.9 mg/dL) and ESR (51 mm/h). Blood count, renal, thyroid and liver function without changes. Urine exam without sediment. Requested ANA and anti-dsDNA, nonreactive. An echocardiogram was performed with the presence of a laminar pericardial effusion (2.5 mm), with no changes in function or the presence of areas of hypokinesia. Diuretic therapy was started with improvement of the complaints. Hospital discharged; however, symptoms recurred, initiating symmetrical and additive arthritis of elbows, knees, and ankles. She maintained a complaint of edema in the distal third of both legs and feet, with hypersensitivity to touch and mild skin thickening. Admitted to the care of Rheumatology and a new investigation was initiated. Echocardiogram, chest computed tomography (CT) and CT angiography scans of the abdomen and pelvis were performed, with no changes. A new autoimmune panel was requested, with ANA 1/160 being evidenced, with a nonspecific pattern, dense fine speckled nuclear, and reagent anticentromere. A hypothesis of edema was raised as a result of the edematous phase of systemic sclerosis, with initiation of corticosteroid therapy and methotrexate, with improvement of the complaints. CONCLUSIONThis report illustrates the onset of an atypical case of the edematous phase of systemic sclerosis, affecting the lower limbs, after a picture of COVID-19 infection. This early stage of the disease is often misdiagnosed, preventing early identification and treatment.