We describe a case of a 48-year-old woman who presented with acute respiratory failure due to diffuse alveolar haemorrhage and acute renal failure due to pauci-immune glomerulonephritis consistent with a new diagnosis of microscopic polyangiitis (MPA). The patient had a recent SARS-CoV-2 infection 6 weeks before MPA diagnosis and had stopped immunosuppression for her rheumatoid arthritis (RA) at that time. The patient was treated with pulse intravenous steroids, plasma exchange therapy and rituximab, which induced remission of her illness. This case highlights a timely dilemma of holding immunosuppression in a RA patient with low disease activity on combination therapy with SARS-CoV-2 infection, and the potential risk of developing an additional autoimmune disease, such as vasculitis, given their existing autoimmunity due to RA.
Anterior chest wall pain is a feature of axial spondyloarthritis that is understudied. It is rarely the presenting symptom, and when present, may suggest severe disease. We present the case of a 35-year-old female with recurring presentations of debilitating chest pain, subsequently diagnosed with axial spondyloarthritis. Awareness of this presentation can lead to earlier diagnosis and treatment of patients presenting with manubriosternal joint involvement as their initial symptom of axial spondyloarthritis.
Granulomatosis with polyangiitis (GPA) is an ANCA-associated, systemic vasculitis of small and medium-sized blood vessels. GPA causes inflammation and destruction to the vessel wall and eventual tissue and organ damage. It classically affects the tissues and vasculature of the sinuses, lungs, and kidneys. The organ damage results in epistaxis, cough, hemoptysis, shortness of breath and/or even kidney failure. Cutaneous manifestations are rare, but have been reported. We discuss the case of a 27-year-old African American female who presented with skin lesions on her bilateral forearms and hands, several years after diagnosis and treatment for GPA. The lesions were isolated papules, located on bilateral elbows, palms, and the lateral edge of the 3rd digit on her left hand. The lesions were intensely pruritic and non-painful. A skin biopsy of the lesions showed neutrophilic and granulomatous inflammation with nuclear debris, a largely non-specific histopathologic finding. An extensive autoimmune work up revealed elevated c-ANCA/PR3-ANCA levels supporting a diagnosis of cutaneous GPA. GPA is diagnosed by a combination of clinical signs and symptoms, serologic testing, and histology from biopsy of affected organs. Patient’s who meet the criteria should be tested for anti-neutrophil cytoplasmic antibody (ANCA), specifically c-ANCA/PR3-ANCA. A positive ANCA is supportive for GPA diagnosis. However, a negative ANCA does not rule out disease. Patients with cutaneous findings suggestive of GPA and positive c-ANCA/PR3-ANCA serologic testing should be closely followed up, which will lead to overall better prognosis, improved health outcomes and reduced patient and health care expenses.
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