Metastatic Carcinoma of Prostate as a Mimicker of SAPHO Syndrome.

Paraneoplastic arthritides are a group of immune-mediated inflammatory arthropathies associated with occult or manifest malignancy. Musculoskeletal spread of an underlying malignancy may also mimic many rheumatologic conditions. Distinguishing primary rheumatologic condition from paraneoplastic arthritides versus direct musculoskeletal spread of malignancy can be challenging especially in individuals with prior history of cancer and new musculoskeletal complaints. SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is an uncommon, although under recognized autoimmune disorder. Two musculoskeletal manifestations, namely inflammatory osteitis and hyperostosis of anterior chest wall with or without dermatologic manifestations, constitute a unifying feature of SAPHO syndrome. However, diagnosis of SAPHO syndrome is one of exclusion, and a wide variety of disorders including infections, malignancy (chondrosarcoma/osteosarcoma/metastasis), metabolic bone disorders (Paget's disease), osteoarthritis, seronegative spondyloarthropathy (spA) and osteonecrosis form part of a broad differential diagnosis. We present the case of a man, aged 72 years, with signs and symptoms of SAPHO syndrome and skin findings. Detailed history, radiological imaging, dermatology appearance, and role of immunohistochemical markers, especially staining for NKX3.1 protein with a novel antibody, led to a diagnosis of metastatic prostate adenocarcinoma. To our knowledge, this is the first case of metastatic adenocarcinoma of the prostate manifesting as SAPHO syndrome and cutaneous metastasis.

Disseminated Blastomycosis Presenting with Spontaneous Coronary Artery Dissection.

Spontaneous coronary artery dissection (SCAD) is increasingly recognized as an important cause of acute coronary syndrome (ACS) and myocardial infarction (MI) in individuals with few or no known atherosclerotic risk factors. While systemic autoimmune inflammatory disorders are associated with precipitating SCAD, the role of infection-induced systemic inflammation in SCAD is not well defined. We present the case of a 49-year-old Caucasian woman with ST-elevation myocardial infarction (STEMI) diagnosed as SCAD from a severe systemic inflammatory response related to disseminated blastomycosis. Punch biopsy of a skin lesion and synovial fluid culture confirmed Blastomyces dermatitidis. This case suggests the possibility of systemic infection-induced inflammation as a precipitating factor in SCAD pathogenesis similar to autoimmune inflammatory disorders. LEARNING POINTS: Recognize the role of systemic inflammation from severe infection as a possible cause of spontaneous coronary artery dissection (SCAD).Recognize that cardiac involvement is rare in blastomycosis.Coronary revascularization may be required in SCAD for haemodynamic instability, ischaemic chest pain progression, and myocardium at risk.

Biologic Agent-Associated Cutaneous Adverse Events: A Single Center Experience.

Biologic agents are regarded as an effective treatment for a variety of autoimmune diseases. These drugs have an acceptable safety and tolerability profile, although an increasing number of autoimmune conditions have been reported with their use. Additionally, a variety of cutaneous diseases have been associated with their use. Here we report our experience of adverse cutaneous events with the use of biologic agents. An alternative explanation for patients presenting with adverse cutaneous events including drug interactions must be carefully investigated.

Fibrillary Glomerulonephritis in Primary Sjogren's Syndrome: A Rare Cause of Renal Failure.

Renal involvement in primary Sjogren's syndrome (pSS) varies in severity and prevalence. Although previously felt to be uncommon, kidneys can be involved in 25% to 30% of pSS patients. Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that can occur in association with another autoimmune condition or malignancy. The diagnosis relies on renal biopsy findings of haphazardly arranged fibrils in all glomerular compartments and distinction from other forms of fibrillary glomerulopathies such as renal amyloidosis and immunotactoid glomerulopathy. FGN responds poorly to immunosuppressive therapy and has a poor prognosis. Here, we describe a case of FGN in a patient with asymptomatic pSS. We describe the diagnostic work-up, clinical course, treatment utilized, and 1-year follow-up. There is one other case in the literature of FGN in a patient with pSS. The rarity of this association and distinction of FGN from other forms of renal involvement in pSS is important as it impacts therapy and prognosis. The case highlights electron microscopy findings in FGN and poor prognosis.

Immune Mediated Necrotizing Myopathy: a Cause of Isolated Myopathy of Neck Extensor Muscle.

Immune mediated necrotizing myopathy (IMNM) is a unique form of myositis that is characterized by distinct muscle biopsy features including abundant myofiber necrosis, degeneration, and regeneration with only minimal, if any, inflammation on muscle biopsy. IMNM is clinically similar to idiopathic inflammatory myopathy (IIM); hence, muscle biopsy is essential to diagnose IMNM. Herein we describe a case of neck extensor weakness due to necrotizing myopathy. Isolated weakness of the neck extensor muscles is uncommon in IIM and IMNM. This case describes the diagnostic work-up, treatments utilized, and 2 year follow-up course without involvement of other muscle groups and without progression of neck extensor muscle weakness. Advanced imaging using magnetic resonance imaging (MRI) facilitated the diagnosis by identifying the affected muscles and site for muscle biopsy.

Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis.

To describe clinical features and outcomes of 26 patients with idiopathic retroperitoneal fibrosis from a single center, we reviewed medical records of consecutive patients with idiopathic retroperitoneal fibrosis evaluated at our facility from January 1, 1998 to December 31, 2013 for clinical features, laboratory and radiographic findings, management, and outcomes. Twenty-six patients met criteria for idiopathic retroperitoneal fibrosis and were included in the study. Median age at diagnosis was 58 years; male-female ratio was 3.3:1.0. Median duration of symptoms was 7 weeks. Abdominal, flank, and/or low back pain were the most common presenting symptoms. Four patients (15 %) had associated autoimmune or fibrosing disorders. Baseline erythrocyte sedimentation rate was elevated in 17 (77 %) of 22 patients tested and C-reactive protein was elevated in 10 (56 %) of 18 patients tested. Hydronephrosis was present in 17 (68 %) patients; 8 (47 %) of 17 had bilateral hydronephrosis. Retroperitoneal mass biopsy was performed in 18 (69 %) patients. Two patients had idiopathic retroperitoneal fibrosis classifiable as IgG4-related disease. Therapy consisted of medications alone in 7 cases, surgical interventions alone in 7 cases, and a combination in 11 cases. One patient achieved remission with no treatment. Most patients treated medically received initial corticosteroids. Methotrexate (1 case), azathioprine (1 case), mycophenolate mofetil (1 case), and tamoxifen (5 cases) were used. No relapses occurred after a median 5-year follow-up. Two (8 %) patients died; five (19 %) developed cancer after diagnosis. In this series, we emphasize the importance of early diagnosis and therapy for overall favorable prognosis of idiopathic retroperitoneal fibrosis.

Antiphospholipid antibodies-associated diffuse alveolar hemorrhage.

OBJECTIVES: To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH). METHODS: We reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes. RESULTS: A total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission. CONCLUSIONS: DAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.

Sjögren's syndrome and neuromyelitis optica spectrum disorders (NMOSD)--a case report and review of literature.

BACKGROUND: Neuromyelitis optica (NMO) is a rare relapsing auto-immune disease of the central nervous system which is sometimes found in association with other autoimmune disorders including Sjogren's syndrome. We present the case of a middle aged female with Sjogren's syndrome (SS) and Neuromyelitis optica spectrum disorders (NMOSD) who had a rapidly declining neurological illness that responded to immunosuppressive therapy. CASE PRESENTATION: A 51-year-old female with Sjogren's syndrome and recent history of varicella zoster infection presented with right upper and lower extremity weakness of one week duration. She was noted to have contrast enhancement at C2-C4 cord levels on cervico-thoracic MRI. Comprehensive work up was negative except for presence of a mild lymphocytic pleocytosis and oligoclonal bands in the CSF. She was diagnosed with transverse myelitis secondary to varicella zoster infection and was treated with high dose steroids in addition to acyclovir with improvement in her symptoms. Two months later she developed left upper and lower extremity weakness, bilateral dysesthesias and urinary incontinence. Repeat MRI of the cervico-thoracic spine revealed worsening enhancement at lower cervical cord levels (C5-7) with extension to T1. CSF analysis was unchanged; however immunological work up was abnormal for elevated NMO-IgG/AQP4 antibody. She was diagnosed with NMOSD and was treated with immunosuppressive therapy. Initially with IV methylprednisone and Cyclophosphamide therapy followed by Mycophenolate mofetil (MMF) maintenance therapy with good response. Repeat MRI 6 months later showed near complete resolution of previous abnormal cord signal changes. CONCLUSION: One needs to recognize the relationship between autoimmune diseases especially SS and NMOSD. The presence of NMO antibody has been associated with a relapsing disease course and a careful follow-up, besides use of remission maintenance agents such as MMF and Azathioprine are important to consider.

Changes in osmol gap in chronic kidney disease: an exploratory study.

AIM: There is no data on osmol gap (OG) in chronic kidney disease (CKD) by stage and limited data on OG in adults on maintenance hemodialysis (HD). We aimed to examine the OG between different stages of CKD and to compare the OG pre- and post-HD in those on maintenance HD. METHODS: We conducted a cross-sectional study of 67 patients. The participants were divided into six groups: Group 1-reference group (normal renal function), Group 2-CKD stage 2; Group 3-CKD stage 3; Group 4-CKD stage 4; Group 5-CKD stage 5 and not on dialysis. Group 6 were subjects on maintenance HD. RESULTS: The means of OG ± standard deviation of Groups 1-6 were 15.25 ± 3.0, 20.73 ± 2.68, 22.85 ± 6.99, 24.11 ± 3.64, 25.15 ± 5.06, and 28.88 ± 3.45, respectively (p < 0.001). In the HD group, the difference between the pre-HD and post-HD OG was statistically significant (p < 0.001). CONCLUSION: There is a statistically significant upward trend for OG as CKD stage increases. The OG is elevated in patients on maintenance HD and is normalized by the HD. OG can be a valuable additional tool to suggest CKD stage and serve as a marker of dialysis adequacy.