Case 238: Spontaneous Pneumothorax Secondary to Intrapulmonary Necrobiotic Rheumatoid Nodule.

History A 54-year-old white woman with a history of rheumatoid arthritis who was taking glucocorticoids and methotrexate presented to the emergency department in December with worsening shortness of breath and chest heaviness for 1 week. She reported additional symptoms of weakness, headache, and arthralgia primarily involving her bilateral hands, wrist, ankles, and feet. She denied experiencing fevers, syncope or presyncope, focal neurologic deficits, chest pain, nausea, vomiting, unintentional weight loss, or recent trauma. Additional medical history included hypertension, asthma, degenerative disk disease, and migraine, all of which were reportedly controlled with medications. This patient had a smoking history of 80 pack-years, but she had quit smoking 2 months prior to presentation. She denied abuse of alcohol or recreational drugs and reported she was up-to-date on her immunizations, including those for pneumonia and flu. Family history was pertinent for breast cancer in her mother, sister, and maternal aunt. The patient reported normal findings at screening mammography and colonoscopy. A physical examination was remarkable for slightly asymmetric breath sounds, which appeared to be diminished on the right side. This patient had multiple joint deformities, most notably in the bilateral metacarpophalangeal joints. Initial electrocardiography findings and cardiac biomarkers were negative. Her complete blood count and basic metabolic profile were unremarkable. Posteroanterior and lateral chest radiographs were obtained in the emergency department. Subsequently, computed tomography (CT) of the chest was performed.

Qualitative Assessment of Diffusion Weighted Imaging and Susceptibility Weighted Imaging of Myeloid Sarcoma Involving the Brain.

BACKGROUND AND PURPOSE: Myeloid sarcoma is a rare form of extramedullary leukemia, which can present with or without systemic leukemia. The purpose of this study was to evaluate characteristic computed tomography (CT) and magnetic resonance imaging (MRI) findings (including diffusion weighted imaging and susceptibility weighted imaging) of myeloid sarcoma involving the brain. MATERIALS AND METHODS: One hundred nine patients with pathologically proven myeloid sarcoma underwent pretreatment CT and MRI, which were retrospectively reviewed. Computed tomography and MRI characteristics reviewed include lesion location, shape, size, architecture, margins, ± multiplicity, ± bone destruction, pattern and degree of enhancement, ± restricted diffusion, and ± susceptibility artifact. RESULTS: Twenty-five patients (14 men, 11 women; mean age, 55 years; range, 9-80 years) met the inclusion criteria. Acute myeloid leukemia with subtypes M3 (44.4%) and M5 (22.2%) were the most common. On unenhanced CT, mean lesion size was 1.9 ± 0.4 cm; 60% were intra-axial hyperdense masses, 8% were intraventricular hyperdense masses, 12% were isodense intra-axial masses, and 20% of cases were extra-axial hyperdense nodular masses. There was no observable intralesional or perilesional calcium. On MRI, mean lesion size was 2.1 ± 0.6 cm. The lesions were isointense (80%) or hypointense (20%) on T1-weighted images with homogeneous (88%) or heterogeneous (12%) enhancement. On fluid-attenuated inversion recovery and T2-weighted images, lesions were hyperintense (96%) or isointense (4%) with mild vasogenic edema. Majority (96%) of cases demonstrated restricted diffusion, whereas only a few (16%) demonstrated susceptibility artifact. CONCLUSIONS: In patients with history of leukemia or myeloproliferative disorder, identification of homogenous mass hyperdense on unenhanced CT, T1 isointense, and T2/fluid-attenuated inversion recovery hyperintense with restricted diffusion and homogenous postcontrast enhancement without significant susceptibility artifact is suggestive of myeloid sarcoma.

Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).