Case Reports in Rheumatology The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. A Curious Case of Proximal Muscle Weakness with Eosinophilic Polymyositis Wed, 14 Sep 2016 14:31:18 +0000 Eosinophilic polymyositis (EPM) is part of a rare disorder, eosinophilic myopathies (EM), which is a form of polymyositis characterized by the presence of eosinophils in muscle biopsy sections and occasionally blood eosinophilia. Herein, we are presenting an interesting case of eosinophilic polymyositis presenting with muscle pain with no other organ systems involved. Ciel Harris, Robert Ali, Julio Perez-Downes, Firas Baidoun, Marianne DeLima, Jaimin Shah, Win Aung, and Raafat F. Makary Copyright © 2016 Ciel Harris et al. All rights reserved. Skin Findings in a Patient with Sjogren’s Syndrome Thu, 08 Sep 2016 17:46:22 +0000 Hypergammaglobulinemic purpura (HGP) is a syndrome constellating recurrent purpura, hypergammaglobulinemia, positive rheumatoid factor (RF), anti-Ro/La antibodies, and elevated erythrocyte sedimentation rate (ESR). We present a case of a 29-year-old female who was diagnosed with Sjogren’s syndrome four years prior to presenting with rash on her lower extremities for a period of 6 months. Skin biopsy at the initial visit was consistent with leukocytoclastic vasculitis and was initiated on treatment for it. Her rash evolved into 2–5 mm scattered purpurae while she was on the treatment and a repeat biopsy showed extravasation of RBCs, a sparse mononuclear infiltrate with deposition of plasma cells, and no evidence of leukocytoclastic vasculitis, thus showing a transition from neutrophilic to mononuclear inflammatory vascular disease which is a rare occurrence. Hypergammaglobulinemic purpura sometimes turns out to be a challenging disease to manage and requires an integrated effort from the primary care doctors, rheumatologist, and dermatologist. Prajwal Boddu, Abdul S. Mohammed, and Sonali Khandelwal Copyright © 2016 Prajwal Boddu et al. All rights reserved. A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy Thu, 08 Sep 2016 09:28:46 +0000 A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy. Naofumi Chinen, Yasushi Koyama, Shinji Sato, and Yasuo Suzuki Copyright © 2016 Naofumi Chinen et al. All rights reserved. Heparin-Related Thrombocytopenia Triggered by Severe Status of Systemic Lupus Erythematosus and Bacterial Infection Tue, 06 Sep 2016 17:35:56 +0000 A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT. Satoshi Suzuki, Shihoko Nakajima, Taiki Ando, Keisuke Oda, Manabu Sugita, Kunimi Maeda, Yutaka Nakiri, and Yoshinari Takasaki Copyright © 2016 Satoshi Suzuki et al. All rights reserved. Severe Primary Raynaud’s Disease Treated with Rituximab Mon, 29 Aug 2016 11:00:08 +0000 Raynaud’s phenomenon refers to reversible spasms of the peripheral arterioles that can be primary Raynaud’s phenomenon (PRP) or secondary Raynaud’s phenomenon (SRP) to underlying connective tissue disease, both of which are characterized by a triphasic color response triggered by cold exposure or stress. PRP is typically a benign disease, whereas SRP may progress into digital ulcers and/or gangrene. Here, we report a case of a 55-year-old female diagnosed with PRP 7 years ago. Treatment with first-line agents, including calcium channel blocker, aspirin, and phosphodiesterase inhibitor, did not control her symptoms, which progressed to digital ulceration and gangrene. There were no symptoms of underlying autoimmune disease or malignancy, and autoimmune, serology, and immunology test results were normal; a biopsy of her left little finger was negative for vasculitis. Development to critical digital ischemia necessitated treatment with intravenous iloprost and heparin infusion followed by angioplasty, which led to a partial improvement. Due to persistent symptoms, rituximab therapy was initiated and two cycles induced a complete resolution of symptoms. Mohammed Shabrawishi, Abdurahman Albeity, and Hani Almoallim Copyright © 2016 Mohammed Shabrawishi et al. All rights reserved. Repository Corticotropin Injection for Treatment of Idiopathic Inflammatory Myopathies Tue, 23 Aug 2016 14:29:41 +0000 Idiopathic inflammatory myopathies are a group of systemic autoimmune diseases that involve inflammation of skeletal muscle. The two most common forms are dermatomyositis and polymyositis, the former of which entails a skin component. There are few approved therapeutics available for treatment of this group of diseases and the first-line therapy is usually corticosteroid treatment. Considering that a large proportion of patients do not respond to or cannot tolerate corticosteroids, additional treatments are required. There are second-line therapies available, but many patients are also refractory to those options. H.P. Acthar® Gel (repository corticotropin injection [RCI]) is a melanocortin peptide that can induce steroid-dependent effects and steroid-independent effects. Herein, we present a series of cases that involved the use of RCI in the management of dermatomyositis and polymyositis. RCI treatments resulted in improvement in three of four patients, despite failure with previous therapies. The use of RCI did not exacerbate any comorbidity and no significant changes in blood pressure, weight, or glycemic control were observed. Overall, these results are encouraging and suggest that randomized, controlled clinical trials applying RCI to dermatomyositis and polymyositis are warranted. Aarat Patel, Georgia Seely, and Rohit Aggarwal Copyright © 2016 Aarat Patel et al. All rights reserved. Coexistence of Ankylosing Spondylitis and Neurofibromatosis Type 1 Sun, 14 Aug 2016 08:17:10 +0000 Ankylosing spondylitis (AS) is a systemic disease primarily characterized by the inflammation of sacroiliac joints and axial skeleton. Neurofibromatosis type 1 (NF1) is a multisystem genetic disease which is characterized by cutaneous findings, most importantly café-au-lait spots and axillary freckling, by skeletal dysplasia, and by the growth of both benign and malignant nervous system neoplasms, most notably benign neurofibromas. In this case report, we present a 43-year-old male with AS and NF1. Baris Gundogdu, Servet Yolbas, Ahmet Yildirim, Murat Gonen, and Suleyman Serdar Koca Copyright © 2016 Baris Gundogdu et al. All rights reserved. Polymyalgia Rheumatica Revealing a Lymphoma: A Two-Case Report Thu, 11 Aug 2016 11:53:20 +0000 Introduction. Polymyalgia rheumatica (PMR) is one of the most common inflammatory rheumatism types in elderly population. The link between cancer and PMR is a matter of debate. Methods. We report two cases of PMR leading to the diagnosis of lymphoma and the growing interest of PET-TDM in this indication. Results. A 84-year-old man known for idiopathic neutropenia presented an inflammatory arthromyalgia of the limb girdle since one month. Blood exams highlighted the presence of a monoclonal B cell clone. Bone marrow concluded to a B cell lymphoma of the marginal zone. He was successfully treated with 0.3 mg/kg/d of prednisone, and response was sustained after 6 months. A 73-year-old man known for prostatic neoplasia in remission for 5 years presented arthromyalgia of the limb girdle since one month. PET-CT revealed bursitis of the hips and the shoulders, no prostatic cancer recurrence, and a metabolically active iliac lymphadenopathy whose pathologic exam concluded to a low grade follicular lymphoma. He was successfully treated with 0.3 mg/kg/d of prednisone. Conclusion. These observations may imply that lymphoma is sometimes already present when PMR is diagnosed and PET-CT is a useful tool in the initial assessment of PMR to avoid missing neoplasia. Frank Verhoeven, Xavier Guillot, Mickaël Chouk, Clément Prati, and Daniel Wendling Copyright © 2016 Frank Verhoeven et al. All rights reserved. Olecranon Bursitis Caused by Candida parapsilosis in a Patient with Rheumatoid Arthritis Tue, 09 Aug 2016 10:16:25 +0000 Septic bursitis is usually caused by bacterial organisms. However, infectious bursitis caused by fungi is very rare. Herein, we present a 68-year-old woman with long-standing rheumatoid arthritis who developed pain, erythema, and swelling of the right olecranon bursa. Aspiration of the olecranon bursa showed a white blood cell count of (41% neutrophils, 30% lymphocytes, and 29% monocytes). Fluid culture was positive for Candida parapsilosis. She was treated with caspofungin 50 mg intravenously daily for 13 days followed by fluconazole 200 mg orally daily for one week. She responded well to this treatment but had recurrent swelling of the bursa. Bursectomy was recommended but she declined this option. This case, together with other reports, suggests that the awareness of uncommon pathogens, their presentation, and predisposing risk factors are important to establish an early diagnosis and prevent long-term complications. Carla F. Gamarra-Hilburn, Grissel Rios, and Luis M. Vilá Copyright © 2016 Carla F. Gamarra-Hilburn et al. All rights reserved. Cutaneous Necrotizing Vasculitis and Leukopenia in a Cocaine User: Is Levamisole the Culprit? Sun, 07 Aug 2016 12:55:32 +0000 Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA) in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies. Her skin biopsy showed fibroconnective tissue with signs of necrosis, acute and chronic inflammation, and thrombus formation. She was diagnosed with levamisole-induced vasculitis and successfully treated with withdrawal of cocaine use and local wound care. Lara El Khoury, Nabil Zeineddine, Richard Felix, and Mark Goldstein Copyright © 2016 Lara El Khoury et al. All rights reserved. A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma Sun, 31 Jul 2016 10:09:57 +0000 Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly. Cody M. Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, and Jean M. Elwing Copyright © 2016 Cody M. Lee et al. All rights reserved. Henoch-Schönlein Purpura with Adalimumab Therapy for Ulcerative Colitis: A Case Report and Review of the Literature Wed, 27 Jul 2016 10:53:46 +0000 Tumor necrosis factor-α (TNFα) inhibitor therapy has signified an important milestone in the fight against many rheumatological disorders and inflammatory bowel disease (IBD). Cutaneous adverse events caused by this class of medications are well known but relatively uncommon. Most reactions are mild and rarely warrant treatment withdrawal. Henoch-Schönlein purpura (HSP) is a disease with cutaneous vasculitis, arthritis, and gastrointestinal and renal involvement that is usually seen in children, though the worst complications are typically seen in adults. We present a case of HSP complicating adalimumab treatment in a patient with ulcerative colitis who had achieved endoscopic remission. We review similar cases reported in the literature and discuss the consequences of these autoimmune diseases. Joseph J. LaConti, Jean A. Donet, Jeong Hee Cho-Vega, Daniel A. Sussman, Dana Ascherman, and Amar R. Deshpande Copyright © 2016 Joseph J. LaConti et al. All rights reserved. Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease Mon, 18 Jul 2016 17:28:15 +0000 Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren’s syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus. In this small population of patients there is no correlation with ANCAs. Most of the patients were treated with aggressive immunosuppression and did well if they were treated early in the course of their disease. One of our patients required renal transplant, but she presented late in the course of her disease, as evidenced by chronicity on her renal biopsy. Whether these patients are overlap of vasculitis and other connective tissue diseases or to be considered as a separate entity is yet to be described. Clinicians must be aware of these presentations because initial presentation can be severe. Supraja Yeturi, Mary Cronin, Adam Robin, Campbell Lorna, and Ann K. Rosenthal Copyright © 2016 Supraja Yeturi et al. All rights reserved. Toxic Epidermal Necrolysis-Like Lesions and Systemic Lupus Erythematosus Possibly Triggered by Sulfasalazine Tue, 12 Jul 2016 11:08:59 +0000 This case report describes a patient with arthritis of the large joints, bilateral sacroiliitis, and positive anti-SSA and anti-dsDNA antibody, who received sulfasalazine and shortly thereafter became critically ill. He developed toxic epidermal necrolysis, hemolytic anemia, lymphopenia, markedly elevated ferritin, and muscle wasting. A diagnosis of systemic lupus erythematosus was made, and mycophenolate mofetil and systemic glucocorticoids brought this severe disease under control. Toxic epidermal necrolysis-like lesions and hemophagocytic syndrome have been reported as manifestations of systemic lupus erythematosus. This patient possibly had spondyloarthritis or an undifferentiated connective tissue disease at presentation, and we suggest, based on the timing of events, that sulfasalazine may have acted as a trigger of the severe disease manifestations. Simon Krabbe, Cigdem Gül, Bjarne Andersen, and Niels Tvede Copyright © 2016 Simon Krabbe et al. All rights reserved. Giant Cell Arteritis: An Atypical Presentation Diagnosed with the Use of MRI Imaging Mon, 04 Jul 2016 10:54:26 +0000 Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries in individuals over the age of 50. It is typically characterised by the granulomatous involvement of large and medium sized blood vessels branching of the aorta with particular tendencies for involving the extracranial branches of the carotid artery. Generally the diagnosis is straightforward when characteristic symptoms such as headache, jaw claudication, or other ischemic complications are present. Atypical presentations of GCA without “overt” cranial ischemic manifestations have become increasingly recognised but we report for the first time a case of GCA presenting as mild upper abdominal pain and generalized weakness in the context of hyponatremia as the presenting manifestation of vasculitis that was subsequently diagnosed by MRI scanning. This case adds to the literature and emphasises the importance of MRI in the evaluation of GCA patients without “classic” cranial ischemic symptoms. Siddesh Shambhu and Lisbet Suarez Copyright © 2016 Siddesh Shambhu and Lisbet Suarez. All rights reserved. Assessment and Surgical Treatment of Calcinosis of the Shoulder Associated with CREST Syndrome Wed, 29 Jun 2016 09:57:19 +0000 We report an unusual case of a 65-year-old lady with CREST syndrome with multiple upper and lower limb calcinosis, who presented with severe shoulder pain and stiffness, with widespread intra- and extra-articular calcinosis, which was refractory to conservative measures. We were able to identify the main cause of her symptoms through serial diagnostic injections as calcific biceps tendinosis. We will discuss her assessment and surgical management and the pathophysiology and various treatment modalities for managing the soft tissue calcinosis in rheumatological diseases. R. Manohara and S. J. Breusch Copyright © 2016 R. Manohara and S. J. Breusch. All rights reserved. Triple Valvulopathy and Jaccoud’s Arthropathy: A Case Report and Literature Review Tue, 28 Jun 2016 15:07:21 +0000 Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful. Ali Naderi Mahabadi, Bassam Alhaddad, and Stanley Ballou Copyright © 2016 Ali Naderi Mahabadi et al. All rights reserved. Recurrent Interstitial Pneumonitis in a Patient with Entero-Behçet’s Disease Initially Treated with Mesalazine Sun, 26 Jun 2016 12:13:15 +0000 A 65-year-old man with entero-Behçet’s disease (BD) being treated with mesalazine was presented to our hospital complaining of dyspnea. Computed tomography (CT) of the chest showed ground-glass opacities and he was initially diagnosed with mesalazine-induced interstitial pneumonitis (IP). Besides the discontinuation of mesalazine, a high dose of oral prednisolone was administered and the patient seemed to recover. However, four months later, dyspnea recurred and repeated CT revealed more extensive pulmonary infiltration despite steroid therapy. After the exclusion of infections, we suspected either a recurrence of mesalazine-induced IP or BD-related IP as a clinical manifestation of BD. The patient was treated with intravenous methylprednisolone and cyclophosphamide, followed by orally administered azathioprine, based on the assumption of underlying vasculitis. Thereafter, his condition improved. BD-related IP is an extremely rare condition with limited reports in the literature. Mesalazine-induced IP is also uncommon but the prognosis is generally good after discontinuation of mesalazine with or without steroid therapy. We discuss an extremely rare case, especially focusing on BD-related IP and mesalazine-induced IP as a potential cause of recurrent IP in a patient with entero-BD. Akihiro Nakamura, Tomoya Miyamura, Brian Wu, and Eiichi Suematsu Copyright © 2016 Akihiro Nakamura et al. All rights reserved. An Autopsy Case of Fulminant Amebic Colitis in a Patient with a History of Rheumatoid Arthritis Sun, 12 Jun 2016 07:49:06 +0000 Generally, amebic colitis is localized around the mucosal membrane and often accompanied by diarrhea and abdominal pain. We describe a patient with a history of rheumatoid arthritis who had received prolonged steroid therapy. The patient complained of breathing difficulties because of rheumatoid lung disease. Although the patient was given antibacterial agent, the symptoms did not improve until death. We did an autopsy and found that he had fulminant amebic colitis, although the patient was not previously examined. Histochemical analysis revealed severe inflammation and full-thickness necrosis of the colon by ameba, suggesting the involvement of ameba in the progression of the overall condition. Naoko Kawabe, Fuyuki Sato, Miho Nagasawa, Masako Nakanishi, and Yasuteru Muragaki Copyright © 2016 Naoko Kawabe et al. All rights reserved. Catastrophic Antiphospholipid Syndrome Tue, 07 Jun 2016 12:49:49 +0000 This paper reports one case of successfully treated patients suffering from a rare entity, the catastrophic antiphospholipid syndrome (CAPS). Management of this patient is discussed in detail. Rawhya R. El-Shereef, Zein El-Abedin, Rashad Abdel Aziz, Ibrahim Talat, Mohammed Saleh, Hanna Abdel-Samia, Amro Sameh, and Mahmoud Sharha Copyright © 2016 Rawhya R. El-Shereef et al. All rights reserved. Undiagnosed Sjögren’s Syndrome Presenting as Mesenteric Panniculitis Sun, 05 Jun 2016 11:12:06 +0000 Mesenteric panniculitis is a rare inflammatory and fibrotic process that affects the small intestine mesentery. It may occur following abdominal surgery or in association with a variety of conditions, including malignancy, infection, and certain autoimmune and inflammatory conditions. Herein, an unusual case of mesenteric panniculitis in a patient with primary Sjögren’s syndrome will be presented. The patient presented with abdominal pain, weight loss, sicca symptoms, fatigue, and arthralgia. An abdominal CT revealed mesenteric fat stranding and prominent lymph nodes of the small intestine mesentery. She was found on laboratory workup to have positive antinuclear and anti-SSa antibodies. Minor salivary gland lip biopsy revealed focal lymphocytic sialadenitis. The patient’s symptoms and CT findings improved with corticosteroids. This case suggests that Sjögren’s syndrome should be considered as an underlying disease process in the evaluation of patients with mesenteric panniculitis. Rebecca L. Burns and Sharukh J. Bhavnagri Copyright © 2016 Rebecca L. Burns and Sharukh J. Bhavnagri. All rights reserved. Systemic Lupus Erythematosus Presenting with Massive Ascites: A Case of Pseudo-Pseudo Meigs Syndrome Sun, 05 Jun 2016 05:57:23 +0000 The case presented is consistent with the phenomenon known as Pseudo-Pseudo Meigs Syndrome (PPMS). In it, we describe a young woman with newly diagnosed Systemic Lupus Erythematosus presenting with ascites, pleural effusions, and an elevated CA-125 level. Although rare, and of uncertain etiology, PPMS is becoming increasingly recognized in the literature. It should be considered as a differential diagnosis in such patients, along with the search for malignancy. S. McVorran, J. Song, V. Pochineni, and A. Abrudescu-Opran Copyright © 2016 S. McVorran et al. All rights reserved. HIV Infection and Osteoarticular Tuberculosis: Strange Bedfellows Thu, 26 May 2016 09:35:22 +0000 We report the case of a 47-year-old female patient with rheumatoid arthritis and HIV infection presenting with a 3-week history of a painful swollen knee, increased serum inflammatory markers, and a low CD4 lymphocyte count. The diagnosis of TB arthritis was made by synovial fluid culture, GeneXpert/PCR, and confirmed by histopathology of a synovial biopsy. A mini literature review suggests that although HIV infection is associated with extrapulmonary TB, osteoarticular TB is a relatively unusual presentation in an HIV positive patient. The diagnostic utility of the GeneXpert test is explored. We also describe the patient’s good response to an intra-articular corticosteroid injection in combination with standard anti-TB therapy. B. Hodkinson, N. Osman, and S. Botha-Scheepers Copyright © 2016 B. Hodkinson et al. All rights reserved. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis Wed, 18 May 2016 11:52:51 +0000 In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature. Mohammad E. Naffaa, Alexander P. Rozin, Netanel Horowitz, Ofer Ben-Itzhak, Yolanda Braun-Moscovici, and Alexandra Balbir-Gurman Copyright © 2016 Mohammad E. Naffaa et al. All rights reserved. Development of Eosinophilic Fasciitis during Infliximab Therapy for Psoriatic Arthritis Wed, 11 May 2016 14:11:58 +0000 Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue surrounding muscles, nerves, and blood vessels. While its pathogenesis is not entirely understood, this disorder is thought to be autoimmune or allergic in nature. We present here a case of a 59-year-old male who developed peripheral eosinophilia and subsequent eosinophilic fasciitis during treatment with infliximab. To our knowledge, eosinophilic fasciitis has not been previously described in patients during treatment with an inhibitor of tumor necrosis factor α. Richard Hariman, Payal Patel, Jennifer Strouse, Michael P. Collins, and Ann Rosenthal Copyright © 2016 Richard Hariman et al. All rights reserved. Pneumatosis Intestinalis Associated with Juvenile Dermatomyositis Sun, 08 May 2016 12:27:13 +0000 We herein report a case of pneumatosis intestinalis (PI), a condition characterized by the presence of gas within the wall of the digestive tract, associated with juvenile dermatomyositis (JDM). A 16-year-old girl, diagnosed with JDM at the age of 10, presented with abdominal pain and distention. She developed PI based on radiological findings that also included a dilated large intestine, extraluminal gas, and secondary diaphragmatic elevation. She was observed with medical therapy including bowel rest and hyperbaric oxygen therapy. However, she ultimately developed a strangulated obstruction 5 years after presentation with PI and large intestine resection and colostomy were performed emergently. Takako Miyamae, Naoko Ishiguro, Maria Yonezawa, Katsutoshi Tokushige, and Hisashi Yamanaka Copyright © 2016 Takako Miyamae et al. All rights reserved. Concomitant Guillain Barre Syndrome and Transverse Myelitis as Initial Neuropsychiatric Manifestation in a Case of Lupus: A Diagnostic Quandary Sun, 08 May 2016 09:16:56 +0000 Neuropsychiatric manifestations of systemic lupus erythematosus are varied. Presently nineteen in number, they are classified as whether affecting the central or the peripheral compartments of the nervous system. Its diagnosis however remains difficult, more so when two or more of the syndromes are found concomitantly in the same patient and when they occur in absence of the more classical rash, serositis, and haematological manifestations. We present a case of lupus where myelopathy as well as demyelination existed simultaneously as the initial neurologic manifestation. Anshuman Srivastava, Bijit Kumar Kundu, and Diwakar Kumar Singh Copyright © 2016 Anshuman Srivastava et al. All rights reserved. Fever, Myositis, and Paralysis: Is This Inflammatory Myopathy or Neuroinvasive Disease? Mon, 28 Mar 2016 11:28:35 +0000 West Nile virus (WNV) is a mosquito-borne RNA Flavivirus which emerged in North America in 1999. Most patients present with a febrile illness but a few develop WNV neuroinvasive disease. Myopathy is an uncommon manifestation. We describe a case of a 42-year-old male from Los Angeles who presented with 8 days of fever and muscle pain. Initial physical exam was normal except for 4/5 muscle strength testing in his extremity proximal muscles. Laboratory revealed a creatine kinase of 45,000 and a urinalysis with large blood but no red blood cells, suggesting rhabdomyolysis. The patient’s condition declined despite aggressive supportive care and hydration, and on hospital day #6 he developed severe altered mental status and progressed to complete right arm paralysis and 2/5 muscle strength in bilateral legs. EMG/NCS showed sensorimotor axonal polyneuropathy and the cerebrospinal fluid was positive for IgM and IgG WNV antibodies. The patient was diagnosed with WNV neuroinvasive disease, poliomyelitis (and encephalitis) type with myopathy/muscle involvement. He was treated supportively and his muscle and neurologic disease gradually improved. At 12-month follow-up his muscle enzymes had normalized and his weakness had improved to 5/5 strength in bilateral legs and 3/5 strength in the right arm. Aneeta R. Kiran, Richard A. Lau, Kim M. Wu, Andrew L. Wong, Philip J. Clements, and Emil R. Heinze Copyright © 2016 Aneeta R. Kiran et al. All rights reserved. Combined Thenar and Hypothenar Hammer Syndromes and Raynaud’s Phenomenon Successfully Treated with Iloprost Tue, 22 Mar 2016 15:58:57 +0000 Thenar and hypothenar hammer syndromes are uncommon conditions characterised by digital ischemia of the hand as a result of repetitive trauma at level of the thenar and/or hypothenar eminence and damage to the radial and/or ulnar arteries, respectively. The symptoms are related to the mechanism of the trauma and a Raynaud’s phenomenon can be predominant for a long time. The angiography is the “gold standard” imaging technique which allows to confirm the diagnosis. Therapeutic strategy depends on the type of the lesion and severity of symptoms and includes pharmacological (antithrombotic and thrombolytic drugs) and surgical treatments. The authors present a case of a 53-year-old man, carpenter by profession, with combined thenar and hypothenar hammer syndromes and Raynaud’s phenomenon, successfully treated with a short course of intravenous infusion of iloprost. Alessandro Ciapetti, Marina Carotti, Marco Di Carlo, and Fausto Salaffi Copyright © 2016 Alessandro Ciapetti et al. All rights reserved. Herpes Zoster Meningitis Complicating Combined Tocilizumab and Cyclosporine Therapy for Adult-Onset Still’s Disease Tue, 22 Mar 2016 11:40:02 +0000 A 56-year-old female with refractory adult-onset Still’s disease presented with ocular herpes zoster infection during TCZ treatment. After three days of acyclovir treatment (5 mg/kg), she developed a severe headache and high fever. Viral DNA isolation and cerebral spinal fluid abnormalities led to a herpes zoster meningitis diagnosis. Her meningitis was cured by high doses of intravenous acyclovir (10 mg/kg for 14 days). To our knowledge, this is the first report of meningeal herpes zoster infection in rheumatic diseases under TCZ treatment. Shinichiro Tsurukawa, Nozomi Iwanaga, Yasumori Izumi, Atsunori Shirakawa, Chieko Kawahara, Tetsuo Shukuwa, Miwako Inamoto, Atsushi Kawakami, and Kiyoshi Migita Copyright © 2016 Shinichiro Tsurukawa et al. All rights reserved.