An Unusual Presentation of Adult-Onset Still's Disease in a Patient with Recurrent Pleural and Pericardial Effusions.

Adult-Onset Still's Disease (AOSD) usually presents with a salmon-colored skin rash and arthralgias. However, it can also be present with pleural and pericardial effusions. These effusions are often misdiagnosed as having an infectious etiology because AOSD usually present with fever, leukocytosis, elevated inflammatory markers, procalcitonin and CRP. There is usually a delay in giving steroids until the exclusion of all infectious etiologies, including extensive workups. Herein, we present a case report of AOSD in a patient with recurrent pleural and pericardial effusions, with no skin rashes or joint pain. Patient initially presented with fever, pleural and pericardial effusions, which was then treated as pneumonia with parapneumonic effusions. Patient returned for the second time with shortness of breath, productive cough, and fever, with no resolutions of pleural and pericardial effusions. Patient was found to have an extremely high ferritin levels, whereby a diagnosis of AOSD was made after excluding infection, malignancy and other rheumatological disorders based on the Yamaguchi criteria. AOSD is a rare disease with unusual presentation and diagnosis is often delayed. This case aimed to raise awareness among physicians of the multifaceted presentation of AOSD.

A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing inflammation of the small vessels. Central nervous system (CNS) ANCA-associated vasculitis is a rare manifestation of AAV. Three mechanisms of AAV affecting the CNS have been reported which include contiguous granulomatous invasion from nasal and paranasal sinuses, remote granulomatous lesions, and vasculitis of small vessels. Chronic hypertrophic pachymeningitis (CHP) is the meningeal-site involvement in AAV caused by granulomatous inflammation in the dura mater. We present a case of pachymeningitis manifested with slowly progressive cognitive dysfunction, leptomeningeal enhancement on MRI, and necrotic vessels with surrounding inflammation on biopsy. This case represents a rare development of subsequent CNS AAV in a patient with ANCA-associated interstitial lung disease treated with rituximab with a resolution of leptomeningeal enhancement on a follow-up magnetic resonance imaging (MRI).

Dermatomyositis-Induced Rhabdomyolysis With Features of Necrotizing Myopathy and Acute Inflammatory Demyelinating Polyneuropathy in an Epstein-Barr Virus Infected Patient.

Dermatomyositis (DM) is an autoimmune inflammatory myopathy characterized by features of a typical rash, proximal muscle weakness, and evidence of muscle inflammation. Acute inflammatory demyelinating polyneuropathy (AIDP) is an autoimmune peripheral nerve disease characterized by myelin damage and progressive areflexic weakness and sensory changes. AIDP can be precipitated by viral infections such as Epstein-Barr virus (EBV). We present a case of DM with rhabdomyolysis and necrotizing features, along with AIDP in the setting of EBV viremia. DM and AIDP rarely coincide together. The patient was treated with a combination therapy of methylprednisolone, azathioprine, and intravenous immunoglobulins (IVIGs), which led to significant improvement in his symptoms.