Utility of Coronary Calcium Scoring (CCS) in Connective Tissue Disorders (CTDs) for the Evaluation of Subclinical Coronary Atherosclerosis - A Systematic Review.

OBJECTIVE: To assess the current state of knowledge for the utility of coronary calcium scoring (CCS) in connective tissue disorders (CTDs) as it relates to the presence and quantification of coronary atherosclerosis. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a literature search via PubMed, Embase, Scopus, Web of Science Core Collection, CINAHL, and Cochrane Database of Systematic Review retrieved 1019 studies (since database inception on May 7, 2018) from which 121 manuscripts were eligible for review. Inclusion criteria consisted of studies that investigated CCS in adults with respective CTDs. Studies were excluded if a complete manuscript was not written in English or was a case report. RESULTS: Thirty-one studies were included (27 with healthy age-/gender-matched control group for comparison and 4 without). CTDs analyzed in articles with control group: 11 rheumatoid arthritis (RA), 14 systemic lupus erythematosus (SLE), 4 systemic sclerosis (SSc), 1 idiopathic inflammatory myopathies (IIM), 1 Takayasu arteritis, and 1 psoriasis. Nine out of 11 RA studies, 12 out of 14 SLE studies, and 2 out of 4 SSc studies showed statistically significant increased CCS when compared with the control group. CTDs analyzed in studies without control group: two Kawasaki disease, one juvenile idiopathic arthritis (JIA), and one antiphospholipid syndrome (APS) article, which demonstrated increased coronary arterial calcium burden, however, without statistically significant data. CONCLUSION: CTDs, especially SLE and RA, are associated with higher CCS compared with the control group, indicating increased risk of coronary atherosclerosis. Our search did not elicit sufficient publications or statistically significant results in many other CTDs.

The Central Nervous System Effects and Mimicry of Common Variable Immunodeficiency (CVID): A Case Report with Literature Review.

There is a scarceness of information on the central nervous system effects of common variable immunodeficiency (CVID). A 30-year-old woman with a history of recurrent upper respiratory infections, vitiligo, and immune thrombocytopenic purpura presented with right-sided numbness. Magnetic resonance imaging (MRI) of the thoracic spine revealed a signal hyperintensity. MRI of the brain demonstrated FLAIR hyperintensity in the right middle frontal gyrus. Cerebral spinal fluid was unremarkable. Serum immunoglobulins revealed hypogammaglobulinemia. Endobronchial and subsequent mediastinum biopsies were all negative for sarcoidosis and malignancy. No infectious etiology was found. She was treated with glucocorticoids and intravenous immunoglobulin (IVIG) replacement therapy for CVID-associated myelitis. Follow-up MRI showed improvement; however, her numbness persisted despite these treatments, which led to an outside physician adding methotrexate for their suspicion of sarcoidosis. Her symptoms remained stable for two years, but when the methotrexate dose was weaned, her numbness worsened. Upon review, the treatment team refuted the diagnosis of sarcoidosis but continued treatment with prednisone, IVIG, and methotrexate for CVID-associated myelitis, from which her symptoms have stabilized. Here, we discuss CVID-associated neurological complications, its similarities to sarcoidosis, and a literature review with treatment regimens and outcomes.

Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review.

Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment. The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously. A 33-year-old woman with a history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash. She had acute severe hypertension, diffuse abdominal tenderness, and petechial rash. Diagnostic work-up revealed active urine sediment with proteinuria and hematuria and elevated creatinine, anemia, and thrombocytopenia. She was diagnosed with acute lupus nephritis and early microangiopathic hemolytic anemia in the setting of hypertensive urgency and started on intravenous methylprednisolone 500 mg once a day. Within 48 hours, she developed shock with multiorgan dysfunction and succumbed to her illness. Laboratory tests later showed ADAMTS13 activity less than 10% consistent with TTP and p-antineutrophil cytoplasmic antibody (ANCA) positivity. Autopsy revealed small-vessel vasculitis of the visceral organs. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis. This case illustrates the occurrence of SLE nephritis, p-ANCA vasculitis, and severe TTP with rapidly fatal course, and the importance of having a low threshold for initiating plasma exchange therapy. Here, we discuss the case and provide a literature review on cases of TTP with SLE and vasculitis.

A Case of Gastric Metastatic Melanoma 15 Years after the Initial Diagnosis of Cutaneous Melanoma.

Melanoma is the most common cancer to metastasize to the gastrointestinal tract; however, metastasis to the stomach is a rare occurrence. We present the case of a patient with a history of melanoma of the chest wall 15 years prior to presentation who initially presented to the hospital with sepsis but was later found to have metastatic melanoma in the gastric cardia. This case illustrates the rare occurrence of metastatic melanoma to the stomach which occurred 15 years after the initial skin diagnosis of melanoma was made, its endoscopic appearance, and how the nonspecific symptoms frequently lead to a delayed diagnosis or one that is not made at all until after autopsy. For these reasons, endoscopy should be promptly performed if there is a suspicion of gastrointestinal metastatic melanoma.

Rheumatoid Meningoencephalitis: A Feared Condition in the Era of TNF Blockers.

Central nervous system (CNS) involvement in rheumatoid arthritis (RA) is uncommon, and most of the times, it is secondary to vasculitic processes or extra-articular rheumatoid nodules. Meningeal involvement is extremely rare. In the literature, there are a few case reports, series, and retrospective studies which have demonstrated the association of rheumatoid (aseptic) meningoencephalitis after starting tumor necrosis factor (TNF) inhibitors. We present a case of rheumatoid meningoencephalitis (RME) in a 52-year-old male with a history of RA on adalimumab who presented with headaches associated with motor and sensory deficits, all of which resolved after this diagnosis was achieved and received appropriate therapy with high-dose glucocorticoids. We also present an update with high yield points summarizing clinical features, diagnostic workup, and management of RME. Finally, we post a literature review of relevant CNS rheumatoid disease in patients with and without exposure to TNF inhibitors.