Anti-Neutrophil Cytoplasmic Antibodies: To Care or Not to Care.

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis is a disease process with a wide range of presentations, from asymptomatic or minimally symptomatic disease with positive laboratory testing, to florid acute end-organ damage. Consensus has not been established as to the frequency and/or protocol by which ANCA testing should be repeated. We present the case of a 53-year-old woman who initially came to medical attention with persistent dyspnea and pulmonary infiltrates presumed to be due to acute exacerbation of chronic diastolic congestive heart failure. Extensive infectious disease testing was negative, but ANCA testing was positive. However, because antinuclear antibody (ANA) interference in the original sample rendered the test result difficult to interpret, the test was not repeated. The patient presented eight months after the initial hospitalization with acute hypoxemic respiratory failure requiring intubation, with an ANCA titer of 1:1280 with a negative ANA titer, and renal biopsy-proven severe crescentic glomerulonephritis. In the discussion of our case, we review the importance of interpreting ANCA testing in the correct clinical context. The ANCA laboratory testing requires cautious interpretation, and diagnosed ANCA-associated vasculitis (AAV) requires vigilance for prompt and proactive treatment.

The Sweet syndrome as a cutaneous manifestation of Crohn's disease.

Crohn's disease is an inflammatory bowel disease associated with many extraintestinal manifestations involving multiple organs, including the skin, eyes, liver, and joints. Classic Sweet's syndrome is a cutaneous manifestation of Crohn's disease, characterized by sudden onset of painful plaques or nodules of the skin associated with fever and neutrophilia. We present a case of classic Sweet's syndrome in a patient with well-controlled Crohn's disease.

An Unusual Presentation of Kikuchi-Fujimoto Disease with Recurrent Subdural Effusion.

A 24-year-old man complained of a right temporal headache for four weeks. The patient denied any trauma or previous anticoagulation use. He also reported tender right facial swelling. His physical exam was unrevealing except for right cranial nerve (CN) VI palsy, right parotid enlargement, and cervical adenopathy. Laboratory findings were significant for mild leukopenia at 3300 cells/uL. The computed tomography (CT) scan obtained showed a chronic left subdural effusion with a 4 mm midline shift and confirmed right parotid enlargement and cervical lymphadenopathy. Surgical burr hole evacuation was done and the fluid was sent for wound culture analysis. The infectious diseases service recommended initiating antibiotics, which were later stopped due to cerebrospinal fluid (CSF) cultures with no growth of any organisms. His CN VI palsy resolved during admission. The patient was discharged with follow-up for biopsy. The patient was lost to follow-up. The patient presented to the emergency department (ED) three months later, with a left-sided frontal headache. A repeat CT scan showed a new, right-sided fluid collection outside the brain parenchyma. Burr hole evacuation was done again and purulent fluid was drained. Antibiotics were held this time, but anti-tuberculous therapy was initiated empirically. The otolaryngology service was consulted and a lymph node biopsy was performed. The pathology showed histiocytic necrotizing lymphadenitis. A dural biopsy was done as well and was consistent with histiocytic necrotizing lymphadenitis involving the dura. Cultures from the subdural fluid did not grow any organism. The patient remained neurologically intact. He improved after surgery was done to drain the fluid and was managed by analgesics. The cultures from the extra-parenchymal fluid collection remained negative for pathogens and tuberculous mycobacteria. The patient was discharged with rheumatology clinic follow-up. He saw the rheumatologist six weeks after the discharge. During his clinic visit, the patient reported no recurrence of headaches, fevers, rash, or joint pain. Our patient had a rare presentation of Kikuchi-Fujimoto disease, in which he had a subdural fluid collection resulting in neurological complications that required surgical intervention.

A Case of IgG4-related Sclerosing Mesenteritis.

A 60-year-old African-American male presented to the emergency department with abdominal pain and distention associated with decreased appetite and weight loss for several weeks. A computed tomography (CT) scan of the abdomen and pelvis showed an 8 cm mesenteric mass with surrounding stranding and poorly defined borders. The patient underwent exploratory laparotomy and complete resection of the mass since the frozen section could not give a definite diagnosis. Histopathology showed fibro-adipose tissue with lymphoid hyperplasia, vague nodular collections of foamy histiocytes with giant cell reaction, marked chronic inflammation, fat necrosis, and prominent sclerosis/fibrosis. Methenamine silver and acid-fast stains were negative for fungal and mycobacterial organisms respectively. Examination of tissue with immunohistostains showed increased immunoglobulin G4 (IgG4)-positive plasma cells. Other features observed were scattered areas of phlebitis, pockets of tissue eosinophilia, and focal storiform fibrosis leading to the diagnosis of IgG4-related sclerosing mesenteritis. The patient did not require steroids after the surgical resection and was disease free at six-month follow up.