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A case of systemic lupus erythematosus associated with longitudinal extensive transverse myelitis, cerebral neutrophilic vasculitis, and cerebritis

Modjinou, Dodji; Gurin, Lindsey; Chhabra, Arpit; Mikolaenko, Irina; Lydon, Eileen; Smiles, Stephen
Systemic Lupus erythematosus (SLE) is an autoimmune dis- ease with multiple clinical presentations and manifestations. Here, we report an intriguing case of a 30-year-old female with full-blown SLE, associated with longitudinal extensive transverse myelitis (LETM) on Magnetic Resonance Imaging (MRI) manifested by lower extremity weakness, neurogenic bladder and bowel, and central nervous system (CNS) lupus clinically manifested by changes in mood and behavior as well as neutrophilic vasculitis and cerebritis on pathology. LETM is a rare complication of SLE; however, what makes this case even more intriguing is that it additionally had cerebral lesions consistent with neutrophilic vasculitis and cerebritis, and that it may all have started at least 10 years prior with nonspecific musculoskeletal manifestations sub - sequently followed by a rash as well as intractable fevers of unknown etiology-much later attributed to her lupus. Although she had a most concerning and dramatic pre- sentation, she, so far, had responded very well to therapy including pulse dose steroids, plasmapheresis, intravenous immunoglobulins (IVIG), cyclophosphamide, and related medications.
PMID: 25986355
ISSN: 2328-5273
CID: 1590742

Increased interleukin-1beta gene expression in peripheral blood leukocytes is associated with increased pain and predicts risk for progression of symptomatic knee osteoarthritis

Attur, Mukundan; Belitskaya-Levy, Ilana; Oh, Cheongeun; Krasnokutsky, Svetlana; Greenberg, Jeffrey; Samuels, Jonathan; Smiles, Stephen; Lee, Sicy; Patel, Jyoti; Al-Mussawir, Hayf; McDaniel, Gary; Kraus, Virginia Byers; Abramson, Steven B
OBJECTIVE: To evaluate whether gene expression profiles could serve as biomarkers of symptomatic knee osteoarthritis (OA) by examining gene expression profiles in peripheral blood leukocytes (PBLs) from patients with OA compared with those from non-OA controls, and to determine whether candidate genomic biomarkers (PBL expression of inflammatory genes) predict an increased risk of disease progression in patients with symptomatic radiographic knee OA. METHODS: Three independent cohorts of patients with knee OA and non-OA control subjects were studied. Two cohorts (a learning cohort and a validation cohort) were recruited at New York University Hospital for Joint Diseases (NYUHJD), and 1 cohort (a validation cohort) was recruited at Duke University Medical Center. PBL gene expression was assessed using Affymetrix microarray and was confirmed by quantitative polymerase chain reaction (qPCR). Radiographic progression at 2 years was assessed in 86 patients. RESULTS: We identified 173 genes that were significantly up-regulated or down-regulated (>/=1.5-fold change) in OA PBLs, at a false discovery rate of 5%. Cluster analysis revealed 2 distinct subgroups among the patients with OA: those in whom the expression of interleukin-1beta (IL-1beta) was increased >/=2-fold compared with controls, and those in whom the expression of IL-1beta was comparable with that in controls. Overexpression of IL-1beta in these OA subclasses was validated using qPCR in all 3 cohorts. Patients with the inflammatory 'IL-1beta signature' had higher pain scores and decreased function and were at higher risk of radiographic progression of OA. CONCLUSION: PBLs from patients with symptomatic knee OA display a characteristic transcriptome profile. Moreover, increased expression of IL-1beta identifies a subset of patients with OA who have increased pain and are at higher risk of radiographic progression of OA
PMID: 21717421
ISSN: 1529-0131
CID: 134740

Peripheral blood leukocytes (PBL) gene expression profiles as biomarkers in patients with human knee osteciarthritis (OA) [Meeting Abstract]

Krasnokutsky, S; Attur, M; Belitskaya-Levy, I; Patel, J; Al-Mussawir, H; Smiles, S; Lee, S; Kraus, V; Kong, SY; McDaniel, G; Abramson, SB
ISSN: 0004-3591
CID: 70764

Long-term risks associated with biologic response modifiers used in rheumatic diseases

Imperato, Anna K; Smiles, Stephen; Abramson, Steven B
PURPOSE OF REVIEW: The introduction of tumor necrosis factor-alpha antagonists in 1998 has had a significant impact on the treatment of rheumatoid arthritis. However, as use of these agents has increased worldwide, infrequent adverse events that were not apparent in pivotal controlled clinical trials required for registration have emerged. RECENT FINDINGS: These adverse events include serious infections, particularly tuberculosis, which may be atypical in presentation. Concern regarding increased risk of lymphoma has also emerged, although it remains unclear whether the risk exceeds that observed in other rheumatoid arthritis patients with comparable disease activity. Development of a systemic lupus erythematosus-like syndrome, which typically abates after discontinuation of the drug, is another rare complication that was further reported during the past year. Finally, additional cases of congestive heart failure and demyelinating syndromes (including cases resembling progressive multifocal leukoencephalopathy) have been reported that appear to be related to the tumor necrosis factor-alpha antagonists. SUMMARY: Additional postmarketing surveillance of these and other serious adverse events is necessary to determine the true risk of their occurrence, and whether a reassessment of the overall risk-benefit of tumor necrosis factor-alpha antagonists will be required
PMID: 15103245
ISSN: 1040-8711
CID: 46027

Aspergillus infection of total knee arthroplasty presenting as a popliteal cyst. Case report and review of the literature [Case Report]

Austin KS; Testa NN; Luntz RK; Greene JB; Smiles S
Fungal infections have only rarely been reported to occur in patients having undergone total knee arthroplasty. This case report documents the first known case of Aspergillus fumigatus as the offending organism. Its initial presentation as a popliteal cyst further reinforces the known association of popliteal cysts and intra-articular knee pathology
PMID: 1402949
ISSN: 0883-5403
CID: 13462


ISSN: 0004-3591
CID: 51853

Subcutaneous thoracentesis utilizing an Ommaya reservoir in amyloid cardiomyopathy [Case Report]

Mitnick HJ; Tunick P; Boyd A; Smiles SA; Choy R
PMID: 3945038
ISSN: 0098-7484
CID: 63009

CT demonstration of rapid improvement of retroperitoneal fibrosis in response to steroid therapy [Case Report]

Smith SJ; Bosniak MA; Megibow AJ; Hulnick DH; Smiles S
A case of retroperitoneal fibrosis with hydronephrosis was treated with cortisone therapy. Computed tomographic images recorded marked and rapid reduction in the size of the soft tissue fibrotic mass and resolution of the hydronephrosis
PMID: 3787872
ISSN: 0171-1091
CID: 43701