Case Reports in Rheumatology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Embolic Stroke as the Initial Manifestation of Systemic Lupus Erythematosus Wed, 22 Jul 2015 08:16:34 +0000 http://www.hindawi.com/journals/crirh/2015/373201/ We present a case of a 21-year-old African-American female with no significant medical history, who presented to the emergency department with a one-week history of blurry and double vision. Ophthalmology evaluation revealed bilateral retinal artery occlusion. Further workup with imaging of the brain was consistent with an ischemic stroke. Hereditary hypercoagulable workup was unremarkable and initial testing for antiphospholipid syndrome was positive. She underwent transesophageal echocardiogram (TEE), which showed severe mitral regurgitation and thickening of mitral valve leaflets consistent with Libman-Sacks endocarditis. Autoimmune workup was positive for IF-ANA, anti-RNP, and anti-Smith antibody. She fulfilled 4/11 of the ACR criteria and met 5 of the SLICC (Systemic Lupus International Collaborating Clinics) criteria for lupus (nonscaring alopecia, thrombocytopenia, positive ANA, and positive anti-Smith and positive anti-phospholipid antibodies). This case highlights the importance of early recognition of underlying connective tissue diseases and timely management of these diseases in young patients with no previous manifestations of diseases. Reshma M. Khan, Rajaie Namas, Sachin Parikh, and Bernard Rubin Copyright © 2015 Reshma M. Khan et al. All rights reserved. A Case of Docetaxel Induced Myositis and Review of the Literature Thu, 16 Jul 2015 14:00:29 +0000 http://www.hindawi.com/journals/crirh/2015/795242/ In phase I and II trials taxane chemotherapeutic agents reported side effects, including myelosuppression, peripheral edema, and fluid retention. With further use of these agents, studies in the late 1980s and early 1990s began to report peripheral neuropathy and proximal muscle weakness as common complaints, the later with unexplained pathophysiology. We report a 65-year-old Hispanic woman with estrogen receptor (ER) and progesterone receptor (PR) positive invasive ductal breast carcinoma who presented with right thigh pain and swelling eight days after her third infusion of docetaxel (a taxane chemotherapeutic) and cyclophosphamide. Laboratory findings were notable for elevation in creatine phosphokinase (CPK), aldolase, and erythrocyte sedimentation rate (ESR); a magnetic resonance imaging (MRI) of her lower extremities showed evidence of bilateral muscle edema involving the anterior compartment muscles of the thighs. A workup to rule out other causes of myositis was negative. Docetaxel was not reintroduced and the patient improved with corticosteroids. Since 2005 this is, to our knowledge, the fifth reported case of docetaxel related inflammatory myositis. Taxanes have been noted to cause disabling but transient arthralgias and myalgias; it is important to consider the possibility of inflammatory myopathy as a possible complication in patients undergoing treatment with these agents. Alexandra Perel-Winkler, Regina Belokovskaya, Isabelle Amigues, Melissa Larusso, and Nazia Hussain Copyright © 2015 Alexandra Perel-Winkler et al. All rights reserved. Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis Thu, 09 Jul 2015 08:19:30 +0000 http://www.hindawi.com/journals/crirh/2015/107275/ According to the American Thoracic Society (ATS)/European Respiratory Society consensus classification, idiopathic interstitial pneumonias (IIPs) include several clinic-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated ILD, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. Ultrasound Lung Comets (ULCs) are an echographic chest-sonography hallmark of pulmonary interstitial fibrosis. We describe the ultrasound (US) findings in the follow-up of a NSIP’s case in rheumatoid arthritis (RA). A. Laria, A. Lurati, and M. Scarpellini Copyright © 2015 A. Laria et al. All rights reserved. Undiagnosed Systemic Lupus Erythematosus Presenting as Hemophagocytic Lymphohistiocytosis Wed, 08 Jul 2015 06:09:48 +0000 http://www.hindawi.com/journals/crirh/2015/748713/ Hemophagocytic Lymphohistiocytosis (HLH) is rarely diagnosed in adults. Incidence is reported as one case per million persons per year. It can be triggered by conditions that affect immune homeostasis as infections, malignancies, and rheumatologic disorders. The following case demonstrates a rare instance in which undiagnosed systemic lupus erythematosus (SLE) presented as HLH. A 28-year-old male presented with progressive weakness and recurrent fevers for 2 months. Vital signs were within normal limits except for temperature of 100.3°F. His exam was unremarkable except for a left cervical scar and malar rash. His labs showed pancytopenia with neutropenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. Hemophagocytosis was present on bone marrow biopsy. All workup for a source of infection was negative. A tentative diagnosis of HLH was made based on clinical presentation and laboratory data. The patient was treated with an HLH protocol. Later, it was determined that his HLH was actually secondary to a primary diagnosis of SLE. The patient was treated for SLE with an immunosuppressive regimen of cyclosporine and dexamethasone, and he improved dramatically. HLH rarely presents due to a rheumatologic condition such as SLE. Physicians should consider testing for SLE in patients diagnosed with HLH. Ahmad K. Rahal, Justin Fernandez, and Christopher Dakhil Copyright © 2015 Ahmad K. Rahal et al. All rights reserved. Severe Rhabdomyolysis without Systemic Involvement: A Rare Case of Idiopathic Eosinophilic Polymyositis Wed, 01 Jul 2015 08:52:12 +0000 http://www.hindawi.com/journals/crirh/2015/908109/ Introduction. Eosinophilic polymyositis (EPM) is a rare cause of rhabdomyolysis characterized by eosinophilic infiltrates in the muscle. We describe the case of a young patient with eosinophilic polymyositis causing isolated severe rhabdomyolysis without systemic involvement. Case Presentation. A 22-year-old Haitian female with no past medical history presented with progressive generalized muscle aches without precipitating factors. Examination of the extremities revealed diffuse muscle tenderness. Laboratory findings demonstrated peripheral eosinophilia and high creatinine phosphokinase (CPK) and transaminase levels. Workup for the common causes of rhabdomyolysis were negative. Her CPK continued to rise to greater than 100,000 units/L so a muscle biopsy was performed which showed widespread eosinophilic infiltrate consistent with eosinophilic polymyositis. She was started on high dose systemic corticosteroids with improvement of her symptoms, eosinophilia, and CPK level. Discussion. This case illustrates a systematic workup of rhabdomyolysis in the presence of peripheral eosinophilia. Many differential diagnoses must be considered before establishing a diagnosis of idiopathic eosinophilic polymyositis. To our knowledge, our case of eosinophilic polymyositis is unique as it presented with severe rhabdomyolysis without another organ involvement. Clinicians should maintain a high index of suspicion for this physically debilitating disease to aid in prompt diagnosis. Ayesha Farooq, Vivek Choksi, Andrew Chu, Dhruti Mankodi, Sameer Shaharyar, Keith O’Brien, and Uday Shankar Copyright © 2015 Ayesha Farooq et al. All rights reserved. Takayasu’s Arteritis with Systemic Lupus Erythematosus: A Rare Association Tue, 16 Jun 2015 07:17:49 +0000 http://www.hindawi.com/journals/crirh/2015/934196/ We report the case of a 24-year-old nondiabetic, nonhypertensive lady with history of fatigue, dyspnoea and limb claudication. She has been diagnosed with Takayasu’s arteritis. Subsequently she developed rash, alopecia, joint pain, and various other laboratory abnormalities which led to a diagnosis of SLE. Takayasu’s arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). The absence of specific SLE markers in patients with TA who subsequently develop SLE suggests that the coexistence of these conditions may be coincidental. The antiphospholipid syndrome in patients with SLE may mimic the occlusive vasculitis of TA. Dhrubajyoti Bandyopadhyay, Vijayan Ganesan, Debarati Bhar, Diptak Bhowmick, Sibnarayan Sasmal, Cankatika Choudhury, Sabyasachi Mukhopadhyay, Adrija Hajra, Manas Layek, and Partha Sarathi Karmakar Copyright © 2015 Dhrubajyoti Bandyopadhyay et al. All rights reserved. A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements Tue, 26 May 2015 06:33:38 +0000 http://www.hindawi.com/journals/crirh/2015/392893/ We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy. Kazuhiko Higashioka, Kenji Yoshida, Kensuke Oryoji, Kazuo Kamada, Shinichi Mizuki, Hiroshi Tsukamoto, Eisuke Yokota, and Koichi Akashi Copyright © 2015 Kazuhiko Higashioka et al. All rights reserved. Acute Osteomyelitis of the Symphysis Pubis after Inguinal Hernia Surgery Mon, 20 Apr 2015 11:27:36 +0000 http://www.hindawi.com/journals/crirh/2015/845867/ Osteomyelitis of pubic symphysis is infectious inflammatory condition of the symphysis pubis and rare complication of surgery around inguinal and groin region. It should be kept in mind in the differential diagnosis of lower pelvic pain and should be sought in cases of pelvic insufficiency fractures. Herein, we present a case of a 55-year-old man with osteomyelitis of the symphysis pubis following inguinal hernia surgery for diagnosis and management of this rare condition. Recep Tekin, Rojbin Ceylan Tekin, Figen Ceylan Cevik, and Remzi Cevik Copyright © 2015 Recep Tekin et al. All rights reserved. Corrigendum to “High-Dose Subcutaneous Immunoglobulins for the Treatment of Severe Treatment-Resistant Polymyositis” Thu, 09 Apr 2015 06:07:20 +0000 http://www.hindawi.com/journals/crirh/2015/436736/ Patrick Cherin, Jean-Christophe Delain, Jean-Charles Crave, and Odile Cartry Copyright © 2015 Patrick Cherin et al. All rights reserved. Granulomatosis with Polyangiitis Presenting as a Choroidal Tumor Wed, 08 Apr 2015 09:28:23 +0000 http://www.hindawi.com/journals/crirh/2015/271823/ Granulomatosis with polyangiitis (GPA) sometimes involves the eye orbit; however, choroidal involvements in GPA had been rarely reported. We report a rare case presenting with a choroidal mass in an 83-year-old Japanese woman who presented with left eye pain. Diagnostic biopsy revealed necrotizing vasculitis with infiltrates of inflammatory cells. Diagnosis was localized granulomatosis with polyangiitis. Combined treatments with corticosteroid plus azathioprine resolved the choroidal mass region. Although treatment with corticosteroid and immunosuppressive agents improves the prognosis of the disease, ocular morbidity is still well recognized. Clinicians should consider a differential diagnosis of GPA in patients with inflammatory choroidal tumors. Taro Masuda, Yasumori Izumi, Hayato Takeshita, Chieko Kawahara, Yoshika Tsuji, Hirokazu Kurohama, Nozomi Iwanaga, Miwako Inamoto, Keiichi Kase, Masahiro Ito, Atsushi Kawakami, and Kiyosi Migita Copyright © 2015 Taro Masuda et al. All rights reserved. Monoarticular Hip Involvement in Pseudogout Mon, 09 Mar 2015 14:11:42 +0000 http://www.hindawi.com/journals/crirh/2015/302389/ Pseudogout is the acutest form of arthritis in the elderly. Although clinical manifestations vary widely, polyarticular involvement is typical mimicking osteoarthritis or rheumatoid arthritis. Monoarticular involvement is relatively rare and is generally provoked by another medical condition. There are reported cases of hip involvement by pseudogout in monoarticular form. However, all of the cases were presented as septic arthritis. In this report, we present a case of monoarticular hip involvement mimicking soft tissue abscess. We confirmed the pseudogout diagnosis after ultrasonographic evaluation of the involved hip joint and pathological and biochemical analysis of synovial fluid analysis. Diagnosis is important to avoid unnecessary medical and surgical treatment in cases of the bizarre involvement of hip in pseudogout. Figen Kocyigit, Ersin Kuyucu, and Ali Kocyigit Copyright © 2015 Figen Kocyigit et al. All rights reserved. Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease Mon, 09 Mar 2015 14:07:25 +0000 http://www.hindawi.com/journals/crirh/2015/324365/ IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD. Teresa Carbone, Ricardo Azêdo Montes, Beatriz Andrade, Pedro Lanzieri, and Luis Mocarzel Copyright © 2015 Teresa Carbone et al. All rights reserved. Bullous Systemic Lupus Erythematosus Associated with Esophagitis Dissecans Superficialis Mon, 02 Mar 2015 11:56:21 +0000 http://www.hindawi.com/journals/crirh/2015/930683/ Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months’ history of multiple blistering pruritic skin lesions involving the face and trunk, a photosensitive rash over the face and neck, swelling of the right neck lymph node, and joint pain involving her elbows and wrist. Her malady was diagnosed as bullous systemic lupus erythematosus based on the immunological workup and biopsy of her skin lesions. The patient also complained of odynophagia and endoscopy revealed esophagitis dissecans superficialis which is a rare endoscopic finding characterized by sloughing of the esophageal mucosa. The bullous disorders typically associated with esophagitis dissecans superficialis are pemphigus and rarely bullous pemphigoid. However, this is the first reported case of bullous systemic lupus erythematosus associated with esophagitis dissecans superficialis. Meera Yogarajah, Bhradeev Sivasambu, and Eric A. Jaffe Copyright © 2015 Meera Yogarajah et al. All rights reserved. Rhabdomyolysis Occurring under Statins after Intense Physical Activity in a Marathon Runner Sat, 28 Feb 2015 07:50:16 +0000 http://www.hindawi.com/journals/crirh/2015/721078/ Statins are widely used in the treatment of hypercholesterolemia and their side effects on muscles are well established. Conversely, data are sparse regarding the safety of this class of drugs in subjects who engage in sports, particularly those who have intense sports activity. We report the case of a marathon runner who presented with acute rhabdomyolysis during competition while being under rosuvastatin treatment. This case raises the question of the need for temporary discontinuation of statin therapy when intense physical activity is planned. Éric Toussirot, Fabrice Michel, and Nicolas Meneveau Copyright © 2015 Éric Toussirot et al. All rights reserved. A Case of Multicentric Carcinoid in a Patient with Psoriatic Spondyloarthropathy Mon, 23 Feb 2015 12:38:30 +0000 http://www.hindawi.com/journals/crirh/2015/179696/ We describe the first case of a patient presenting with multicentric carcinoid occurring in the lung and subsequently in the rectum, with chronic psoriatic arthritis. Although reports have been published regarding carcinoid syndrome occurring alongside rheumatoid arthritis, no reports have been made on such a case. Initial presentation of carcinoid syndrome in this patient was insidious and atypical with few symptoms, including shortness of breath and long standing abdominal bloating. Several years later a sudden change in bowel habit prompted a colonoscopy with biopsy that revealed a carcinoid rectal polyp. The case we report describes a rare presentation of carcinoid syndrome in chronic psoriatic arthropathy. Nabil George, Archana Depala, Laith Al Sweedan, and Kuntal Chakravarty Copyright © 2015 Nabil George et al. All rights reserved. Refractory Rheumatic Disorder: Atypical Postpregnancy Osteoporosis Tue, 17 Feb 2015 11:19:07 +0000 http://www.hindawi.com/journals/crirh/2015/327965/ This is a case report on a young patient with severe osteoporosis that was initially revealed when she presented with polyarthralgia during her second pregnancy. Postpartum, the pain increased and her X-ray did not show any abnormalities. A bone scintigraphy was performed. It indicated an inflammatory rheumatic disorder. Six months after partum, an investigation of right coxalgia revealed a spontaneous basicervical fracture. Given the persistent polyarthralgia, the patient underwent a new scintigraphy, which revealed areas of what looked to be old rib and L1 fractures. A subsequent full body magnetic resonance imaging (MRI) scan revealed signal abnormalities that could indicate multiple lower limb bone fractures. Despite exhaustive biological, radiological, and histological testing, no secondary cause for the osteoporosis was found. The patient was started on teriparatide. We finally concluded that, despite the atypical presentation, the patient was suffering from postpregnancy osteoporosis. It is possible that the frequency of occurrence of this still poorly understood disease is underestimated. Cindy Mourgues, Sandrine Malochet-Guinamand, and Martin Soubrier Copyright © 2015 Cindy Mourgues et al. All rights reserved. Elevated Troponin Serum Levels in Adult Onset Still’s Disease Thu, 12 Feb 2015 13:50:12 +0000 http://www.hindawi.com/journals/crirh/2015/732095/ Adult onset Still’s disease (AOSD) is a rare inflammatory systemic disease that occasionally may affect myocardium. Diagnosis is based on typical AOSD symptoms after the exclusion of well-known infectious, neoplastic, or autoimmune/autoinflammatory disorders. In the case of abrupt, recent onset AOSD, it could be particularly difficult to make the differential diagnosis and in particular to early detect the possible heart involvement. This latter event is suggested by the clinical history of the four patients described here, incidentally observed at our emergency room. All cases were referred because of acute illness (high fever, malaise, polyarthralgias, skin rash, and sore throat), successively classified as AOSD, and they presented abnormally high levels of serum troponin without overt symptoms of cardiac involvement. The timely treatment with steroids (3 cases) or ibuprofen (1 case) leads to the remission of clinicoserological manifestations within few weeks. These observations suggest that early myocardial injury might be underestimated or entirely overlooked in patients with AOSD; routine cardiac assessment including troponin evaluation should be mandatory in all patients with suspected AOSD. Carlo Umberto Manzini, Lucio Brugioni, Michele Colaci, Maurizio Tognetti, Amelia Spinella, Marco Sebastiani, Dilia Giuggioli, and Clodoveo Ferri Copyright © 2015 Carlo Umberto Manzini et al. All rights reserved. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia Thu, 12 Feb 2015 08:40:46 +0000 http://www.hindawi.com/journals/crirh/2015/616787/ Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L) after the second weekly rituximab infusion (375 mg/m2 weekly × 4) given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections. Mariangelí Arroyo-Ávila, Ruth M. Fred-Jiménez, and Luis M. Vilá Copyright © 2015 Mariangelí Arroyo-Ávila et al. All rights reserved. Lymphocutaneous Sporotrichosis during Treatment with Anti-TNF-Alpha Monotherapy Tue, 10 Feb 2015 10:09:48 +0000 http://www.hindawi.com/journals/crirh/2015/614504/ Sporotrichosis is an infectious disease caused by Sporothrix schenckii, a dimorphic fungus isolated for the first time in 1896 by Benjamin Schenck from a 36-year-old male patient presenting lesions on the right hand and arm. The infection generally occurs by traumatic inoculation of soil, plants, and organic matter contaminated with the fungus. Different clinical syndromes are described as a direct consequence of S. schenckii infection, including lymphocutaneous and disseminated forms, although extracutaneous presentations are reported most frequently in AIDS patients. Here we describe the case of a 57-year-old Caucasian male diagnosed in 2004 with ankylosing spondylitis under stable treatment with adalimumab monotherapy (40 mg every other week). During a routine follow-up visit in March 2013, he presented with multiple nodular lesions arranged in a linear fashion along the left hand and forearm. After diagnostic aspiration of the lesions, lymphocutaneous sporotrichosis was diagnosed and appropriate therapy started. Francesco Ursini, Emilio Russo, Christian Leporini, Marilena Calabria, Caterina Bruno, Cesare Tripolino, Saverio Naty, and Rosa Daniela Grembiale Copyright © 2015 Francesco Ursini et al. All rights reserved. Tongue Necrosis as an Initial Manifestation of Giant Cell Arteritis: Case Report and Review of the Literature Sat, 31 Jan 2015 08:41:18 +0000 http://www.hindawi.com/journals/crirh/2015/901795/ Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries that mainly affects the external carotid artery. It is a diagnosis of the elderly that typically presents as low-grade fever, temporal tenderness, claudication of the jaw, and in some patients vision loss. In cases where GCA presents with atypical manifestations, the diagnosis may be more difficult, causing a delay in both diagnosis and treatment and ultimately leading to irreversible complications. In this paper, we present an atypical presentation of GCA with symptoms of neck swelling and lingual pain in an elderly female. These symptoms progressed to bilateral necrosis and eventual dislodgement of the tongue. Lingual necrosis is a severe potential complication in GCA. In patients presenting with lingual swelling, pain, and discoloration, GCA should be suspected and prompt therapy should be initiated to avoid irreversible complications. Jose R. Zaragoza, Natalia Vernon, and Gisoo Ghaffari Copyright © 2015 Jose R. Zaragoza et al. All rights reserved. Nonbacterial Osteitis of the Clavicle: Longitudinal Imaging Series from Initial Diagnosis to Clinical Improvement Tue, 27 Jan 2015 10:58:06 +0000 http://www.hindawi.com/journals/crirh/2015/182731/ Nonbacterial osteitis is a rare autoinflammatory disease. Often it is mistaken for a tumor or osteomyelitis. We present a case of a twelve-year-old girl referred to our hospital because of a lesion of the right clavicle. The differential diagnoses were sarcoma, osteitis, and Langerhans cell histiocytosis. After biopsy the diagnosis nonbacterial osteitis (NBO) was established. Treatment of choice is a nonsteroidal anti-inflammatory drug. This case report gives a complete follow-up of the disease, showing the pitfalls of the diagnosis. E. Roos, M. Maas, S. J. M. Breugem, G. R. Schaap, and J. A. M. Bramer Copyright © 2015 E. Roos et al. All rights reserved. A New Mutation in Blau Syndrome Tue, 27 Jan 2015 07:57:47 +0000 http://www.hindawi.com/journals/crirh/2015/463959/ Blau syndrome is a rare, autosomal dominant, granulomatous autoinflammatory disease. The classic triad of the disease includes recurrent uveitis, granulomatous dermatitis, and symmetrical arthritis. Blau syndrome is related to mutations located at the 16q12.2–13 gene locus. To date, 11 NOD2 gene mutations causing Blau syndrome have been described. Here, we describe a 5-year-old male patient who presented with Blau syndrome associated with a novel sporadic gene mutation that has not been reported previously. Cengiz Zeybek, Gokalp Basbozkurt, Davut Gul, Erkan Demirkaya, and Faysal Gok Copyright © 2015 Cengiz Zeybek et al. All rights reserved. Fatal Cytomegalovirus Disease after Combination Therapy with Corticosteroids and Rituximab for Granulomatosis with Polyangiitis Wed, 14 Jan 2015 11:53:11 +0000 http://www.hindawi.com/journals/crirh/2015/538137/ The association of cytomegalovirus (CMV) with autoimmune disease is poorly understood with suggested causality and reported viral reactivation coinciding with active inflammation. We report a case of a patient who presented with diffuse alveolar hemorrhage and acute renal failure from rapidly progressive glomerulonephritis ultimately diagnosed with granulomatosis with polyangiitis (GPA). She was acutely managed with plasmapheresis to reduce antibody-mediated end-organ damage, hemodialysis for worsening hyperkalemia and acidosis, and high-dose intravenous methylprednisolone. She was transitioned to oral prednisone and started on weekly rituximab with resultant remission induction over a three-week period at which point she developed reactivation of CMV causing severe fatal lung disease and viremia. The case highlights the multiple factors associated with CMV reactivation in cases of severe systemic inflammatory states and the need for further research to help establish practice guidelines regarding antimicrobial prophylaxis in patients with autoimmune diseases on prolonged courses of corticosteroids and biologic agents. Talal Hilal Copyright © 2015 Talal Hilal. All rights reserved. Three Cases of Previous Smokers with Rheumatoid Arthritis Who Did Not Respond to Tumor Necrosis Factor Inhibitors Were Treated Successfully with an Anti-Interleukin-6 Receptor Antibody Wed, 14 Jan 2015 08:05:25 +0000 http://www.hindawi.com/journals/crirh/2015/806725/ We report three cases of previous smokers who did not respond to TNF inhibitors but who responded successfully to an anti-interleukin-6 receptor antibody (tocilizumab (TCZ)). Case 1 is a 63-year-old woman whose smoking index was 200 and had been complaining of polyarthralgia since 1996. She started treatment with etanercept due to high disease activity, but her DAS28-CRP was 4.2. She was therefore switched to TCZ, which dramatically improved her symptoms; her DAS28-CRP had decreased to 2.1. Case 2 is a 64-year-old man whose smoking index was 1600 and had been complaining of polyarthralgia since 2006. Because his DAS28-CRP score increased over time to 5.9, etanercept and adalimumab were added sequentially, but he showed no response over the course of two years. The patient was therefore switched to TCZ, which dramatically improved his symptoms: his DAS28-CRP decreased to 2.7. Case 3 is a 48-year-old woman whose smoking index was 560 and had been complaining of pain in both knee joints since 2001. She was treated with adalimumab due to high disease activity but showed no response over the course of 1.5 years. The patient was therefore switched to TCZ, and her DAS28-CRP decreased to 1.8. An IL-6 blockade might be suitable for treating these 3 cases of previous smokers. Yasuo Iwata Copyright © 2015 Yasuo Iwata. All rights reserved. Unilateral Heliotrope Rash in Juvenile Dermatomyositis: An Unusual Presentation of an Underlying Serious Disease Mon, 22 Dec 2014 00:10:12 +0000 http://www.hindawi.com/journals/crirh/2014/979856/ Background. Heliotrope rash is one of the characteristic skin manifestations of juvenile dermatomyositis. It is a reddish-purple rash on the upper eyelids that is usually bilateral. Case Presentation. We report a boy who presented with unilateral heliotrope rash, Gottron’s papules, and muscle weakness. Muscle biopsy was consistent with inflammatory myositis. Patient was started on prednisolone and methotrexate with an excellent response in both the skin and muscles. Conclusion. Unilateral heliotrope rash can occur in patients with juvenile dermatomyositis. Being a paraneoplastic condition caution should be taken not to miss any underlying malignancy. Ghada Al-Janobi, Hisham Alkhalidi, and Mohammed A. Omair Copyright © 2014 Ghada Al-Janobi et al. All rights reserved. Gout Initially Mimicking Rheumatoid Arthritis and Later Cervical Spine Involvement Tue, 09 Dec 2014 07:22:23 +0000 http://www.hindawi.com/journals/crirh/2014/357826/ Gout is clinically characterized by episodes of monoarthritis, but if not treated properly, it can lead to a chronic polyarthritis, which may eventually mimic rheumatoid arthritis (RA). We present the case of a 59-year-old man, with a history of symmetrical polyarthritis of the large and small joints with later development of subcutaneous nodules, which was initially misdiagnosed as RA, being treated with prednisone and methotrexate for a long period of time. He complained of occipital pain and paresthesia in his left upper limb, and computed tomography (CT) and magnetic resonance imaging (MRI) revealed the presence of an expansive formation in the cervical spine with compression of the medulla. He was admitted for spinal decompressive surgery and the biopsy specimen demonstrated a gouty tophus. Chronic gout can mimic RA and rarely involves the axial skeleton, and thus its correct diagnosis and the implementation of adequate therapy can halt the development of such damaging complications. Eduardo Araújo Santana Nunes, Adroaldo Guimarães Rosseti Jr., Daniel Sá Ribeiro, and Mittermayer Santiago Copyright © 2014 Eduardo Araújo Santana Nunes et al. All rights reserved. Transient Antiphospholipid Syndrome Associated with Primary Cytomegalovirus Infection: A Case Report and Literature Review Mon, 08 Dec 2014 07:13:29 +0000 http://www.hindawi.com/journals/crirh/2014/271548/ Viral infection is known to induce transient autoimmunity in humans. Acute cytomegalovirus (CMV) infection is implicated in occasional thrombosis formation. We here, for the first time, report a 19-year-old female who had an acute CMV infection, leading to a deep venous thrombosis and a pulmonary embolism along with transient appearance of lupus anticoagulant. The pathological role of antiphospholipid antibodies in CMV-mediated thrombosis is discussed. Tsuyoshi Nakayama, Mitsuteru Akahoshi, Kensuke Irino, Yasutaka Kimoto, Yojiro Arinobu, Hiroaki Niiro, Hiroshi Tsukamoto, Takahiko Horiuchi, and Koichi Akashi Copyright © 2014 Tsuyoshi Nakayama et al. All rights reserved. A Challenging Twist in Pulmonary Renal Syndrome Thu, 27 Nov 2014 07:32:12 +0000 http://www.hindawi.com/journals/crirh/2014/516362/ Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09 mg/dL (baseline 0.9). Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed. Rajaie Namas, Bernard Rubin, Wamidh Adwar, and Alireza Meysami Copyright © 2014 Rajaie Namas et al. All rights reserved. IgG4-Related Disease: A Multispecialty Condition Mon, 24 Nov 2014 13:02:00 +0000 http://www.hindawi.com/journals/crirh/2014/723493/ IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases. Iuri Usêda Santana, Emanuela Pimenta da Fonseca, and Mittermayer Barreto Santiago Copyright © 2014 Iuri Usêda Santana et al. All rights reserved. Posterior Reversible Encephalopathy Syndrome in a Patient with Systemic Lupus Erythematosus/Systemic Sclerosis Overlap Syndrome Thu, 20 Nov 2014 09:29:12 +0000 http://www.hindawi.com/journals/crirh/2014/862570/ Posterior reversible encephalopathy syndrome is a clinicoradiologic entity associated with diverse medical conditions. It is very important to properly recognize this condition because early diagnosis and treatment usually result in its complete resolution, whereas a delay in giving an adequate therapy may lead to permanent neurologic sequelae. A case of posterior reversible encephalopathy syndrome in a female patient with an overlap syndrome of systemic lupus erythematosus and systemic sclerosis is presented here. Juliana Gomez, Erik J. Jhonston, and Francisco Zevallos Copyright © 2014 Juliana Gomez et al. All rights reserved.