Case Reports in Rheumatology The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Acute Hemorrhagic Myositis in Inflammatory Myopathy and Review of the Literature Tue, 14 Oct 2014 12:49:38 +0000 We describe two patients with dermatomyositis that presented with interstitial lung disease, positive V and Shawl sign who developed acute spontaneous abdominal/retroperitoneal bleed. Both patients expired despite aggressive treatment and resuscitation. Hemorrhagic myositis in these two patients with inflammatory myopathy is a very rare complication. The association of anti-Ro52 with this potentially very serious complication remains unclear. This potential relationship should be further evaluated in future studies. Howard Van Gelder, Kim M. Wu, Nayiri Gharibian, Dharmi B. Patel, Philip J. Clements, Emil R. Heinze, Robert I. Morris, and Andrew L. Wong Copyright © 2014 Howard Van Gelder et al. All rights reserved. Systemic Sclerosis and Silicone Breast Implant: A Case Report and Review of the Literature Tue, 30 Sep 2014 06:28:58 +0000 Environmentally induced systemic sclerosis is a well-recognized condition, which is correlated with exposure to various chemical compounds or drugs. However, development of scleroderma-like disease after exposure to silicone has always been a controversial issue and, over time, it has triggered spirited debate whether there is a certain association or not. Herein, we report the case of a 35-year-old female who developed Raynaud’s phenomenon and, finally, systemic sclerosis shortly after silicone breast implantation surgery. Antonios Psarras, Ioannis Gkougkourelas, Konstantinos Tselios, Alexandros Sarantopoulos, and Panagiota Boura Copyright © 2014 Antonios Psarras et al. All rights reserved. Piriformis Syndrome in Fibromyalgia: Clinical Diagnosis and Successful Treatment Mon, 22 Sep 2014 07:14:16 +0000 Piriformis syndrome is an underdiagnosed extraspinal association of sciatica. Patients usually complain of deep seated gluteal pain. In severe cases the clinical features of piriformis syndrome are primarily due to spasm of the piriformis muscle and irritation of the underlying sciatic nerve but this mysterious clinical scenario is also described in lumbar spinal canal stenosis, leg length discrepancy, piriformis myofascial pain syndrome, following vaginal delivery, and anomalous piriformis muscle or sciatic nerve. In this paper, we describe piriformis and fibromyalgia syndrome in a 30-year-old young lady, an often missed diagnosis. We also focus on management of the piriformis syndrome. Md Abu Bakar Siddiq, Moshiur Rahman Khasru, and Johannes J. Rasker Copyright © 2014 Md Abu Bakar Siddiq et al. All rights reserved. Kikuchi-Fujimoto Disease Associated with Symptomatic CD4 Lymphocytopenia Wed, 17 Sep 2014 10:34:43 +0000 Kikuchi-Fujimoto disease is a rare benign condition of necrotising histiocytic lymphadenitis with unknown aetiology. We describe here a 30-year-old African American female who presented with fever, generalized rash, cervical lymphadenopathy, and oral candidiasis and was found to have Kikuchi-Fujimoto disease on lymph node biopsy with low CD4 count. The initial presentation was concerning for acute retroviral infection. Her HIV serology and HIV RNA PCR were negative however she had low CD4 count with reversal of CD4/CD8 ratio. Although low CD4 count has been associated with autoimmune disease, it has not been described with Kikuchi-Fujimoto disease. We report the first case of Kikuchi-Fujimoto disease associated with symptomatic CD4 lymphocytopenia. Meera Yogarajah and Bhradeev Sivasambu Copyright © 2014 Meera Yogarajah and Bhradeev Sivasambu. All rights reserved. Pneumocystis jirovecii Pneumonia in a Patient with Rheumatoid Arthritis Treated with Abatacept Wed, 17 Sep 2014 07:16:51 +0000 Rheumatoid arthritis (RA) is an autoimmune disease characterized by synovial membrane inflammation and joint cartilage destruction. Abatacept is a biologic agent that blocks the costimulation signals, preventing antigen presentation and proliferation of T lymphocytes. It is approved for the treatment of patients with RA. Pneumocystis jirovecii pneumonia (PJP) is an infectious disease complicating several immunosuppressive drugs. PJP associated with abatacept has not been reported yet in the medical literature. Various factors, such as the mechanism of action of abatacept, may contribute to predisposing to  Pneumocystis jirovecii infection. In this paper, we report a patient with RA who developed PJP under abatacept treatment. Fabio E. Ospina, Andrés Agualimpia, Fabio Bonilla-Abadía, Carlos A. Cañas, and Gabriel J. Tobón Copyright © 2014 Fabio E. Ospina et al. All rights reserved. Vaginoperineal Fistula as a Complication of Perianal Surgery in a Patient with Sjögren’s Syndrome: A Case Report Wed, 10 Sep 2014 07:30:37 +0000 Forty-seven-year-old woman with Sjögren’s syndrome had been operated on because of transsphincteric perianal fistula secondary to perianal abscess. Vaginal wall injury occurred during the course of the operation and injured tissue was repaired primarily. Three months later, patient suffered from the recurrence of perianal fistula symptoms and fistulectomy was performed once again under antibiotic suppression. Several months later, perineal discharge continued, and, therefore, patient was admitted to the hospital for the third time and a fistulotomy was performed. Two months after the third operation, patient was admitted with leukorrhea and a perineovaginal fistula was detected. This time, not only her surgical problem but also her immune system disorder was considered in the preoperative workup. Then, patient was hospitalized for the fourth time and “fistulectomy/perineoplasty” was performed successfully. We believe that patients with autoimmune disorders with or without medical treatment may have healing problems during the course of surgical processes and therefore such medical problems must be taken into consideration by the surgeons. Kemal Beksac, Mert Turgal, Derman Basaran, Omer Aran, and M. Sinan Beksac Copyright © 2014 Kemal Beksac et al. All rights reserved. Relapsing Polychondritis in a Patient with Ankylosing Spondylitis Using Etanercept Tue, 09 Sep 2014 00:00:00 +0000 Relapsing polychondritis (RP) is an autoimmune disease characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues, especially of the ears, nose, joints, and tracheobronchial tree. Its etiology is not well understood, but some studies have linked its pathophysiology with autoimmune disease and autoantibody production. We described a case of a 46-year-old male patient with ankylosing spondylitis who developed RP after the use of etanercept. Few similar cases have been described in the literature. However, they show a possible association between the use of biological inhibitors of tumor necrosis factor (anti-TNFα), which potentially produces autoantibodies, and the development of RP. The treatment was based on data in the literature and included the cessation of biological therapy and the addition of corticosteroids with substantial improvement. Valderilio Feijó Azevedo, Natalia Bassalobre Galli, Alais Daiane Fadini Kleinfelder, Julia Farabolini D’Ippolito, Andressa Gulin Tolentino, and Eduardo Paiva Copyright © 2014 Valderilio Feijó Azevedo et al. All rights reserved. Retracted: Chronic Recurrent Multifocal Osteomyelitis with Concomitant Features of Juvenile Idiopathic Arthritis Thu, 28 Aug 2014 11:33:28 +0000 Case Reports in Rheumatology Copyright © 2014 Case Reports in Rheumatology. All rights reserved. Jaccoud’s Arthropathy in Gout: An Unusual Association Thu, 14 Aug 2014 11:22:58 +0000 Jaccoud’s arthropathy (JA) is a deforming arthropathy observed mainly in patients with systemic lupus erythematosus. We report the case of a 41-year-old Brazilian man with a 10-year history of intermittent monoarthritis followed by bouts of polyarthritis affecting large and small joints. He presented deformities typical of JA and subcutaneous nodules. Histopathological findings of a nodule biopsy were consistent with gouty tophi. To our knowledge, this is the first report of JA secondary to chronic gout. Tayana Donato Alves, Isabela S. Oliveira, Luiza C. Fadul, and Mittermayer B. Santiago Copyright © 2014 Tayana Donato Alves et al. All rights reserved. Pyogenic Sacroiliitis and Pyomyositis in a Patient with Systemic Lupus Erythematous Sun, 03 Aug 2014 06:46:28 +0000 Pyogenic sacroiliitis and pyomyositis are uncommon infectious diseases and their diagnoses are often delayed. They are typically seen in children and young adults and are rare in middle-aged people especially in those affected by rheumatic diseases. We present the first case of a Staphylococcus aureus related pyogenic sacroiliitis associated with iliacus and gluteal pyomyositis occurring in a patient with systemic lupus erythematosus. Antibiotic treatment was administered for a total of 6 weeks with a total recovery. Pyogenic sacroiliitis and pyomyositis, although remaining rare events, should be remembered as severe complications in immunosuppressed patients with inflammatory diseases. Early clinical suspicion, imaging diagnosis, and adequate therapy are decisive for the satisfactory outcome. Wafa Chebbi, Saida Jerbi, Wassia Kessomtini, Asma Fradi, Baha Zantour, and Mohamed Habib Sfar Copyright © 2014 Wafa Chebbi et al. All rights reserved. Takayasu’s Arteritis and Crohn’s Disease in a Young Hispanic Female Thu, 24 Jul 2014 11:41:11 +0000 Takayasu’s arteritis (TA) and Crohn’s disease (CD) are chronic inflammatory granulomatous disorders of undetermined etiology. TA is a large vessel vasculitis with a predilection for the aorta and its branches in young women of Asian descent; whereas CD has characteristic gastrointestinal manifestations more prevalent in young Caucasians. We describe a case of both diseases in a young Hispanic female, review the literature, and impart new insight on possible genetic linkage and the role of interleukin 12 B (IL-12B) as the common autoimmune mechanism and potential therapeutic target in this rare disease combination. Namrata Singh, Shireesh Saurabh, and Irene J. Tan Copyright © 2014 Namrata Singh et al. All rights reserved. High-Dose Subcutaneous Immunoglobulins for the Treatment of Severe Treatment-Resistant Polymyositis Wed, 16 Jul 2014 12:07:35 +0000 Polymyositis is a rare debilitating condition characterized by chronic inflammation and muscle weakness. Standard treatments include corticosteroids and immunosuppressants; however, resistance to these regimens may develop. Intravenous immunoglobulins (IVIg) are thus recommended for patients with drug-resistant polymyositis. The patient presented a resistant polymyositis with severe muscle weakness, increasing dysphagia, and significant loss in weight. Subcutaneous immunoglobulins (SCIg) were initiated after failure of steroids and immunosuppressive drugs. SCIg was given twice per week (2 then 1.3 g/kg/month). Clinical recovery was observed within 2 months after the SCIg initiation. After several injections, the patient showed a progressive improvement in muscle strength. Serum creatine kinase activity decreased to normal levels, and dysphagia was resolved. The SC injections were generally well tolerated and good patient satisfaction was reported. This promising observation suggests that SCIg may be useful in active and refractory polymyositis. Cherin Patrick, Delain Jean-Christophe, Crave Jean-Charles, and Cartry Odile Copyright © 2014 Cherin Patrick et al. All rights reserved. Digital Vasculitis in a Patient with Rheumatoid Arthritis Responded Well to Adalimumab Tue, 15 Jul 2014 11:59:37 +0000 42-year-old old female patient, followed up with diagnosis of rheumatoid arthritis for 15 years, was admitted with necrotising ulcer of left hand 1st and 2nd fingertips and pain, swelling, limitation of movement, and morning stiffness at bilateral wrist, and metacarpophalangeal and proximal interphalangeal joints. Laboratory tests revealed elevated acute phase reactants. Radial and ulnar arteries were clear in upper extremity Doppler ultrasound. The patient was diagnosed as RA activation and digital ulcer and administered iloprost infusion for five days and 1 mg/kg corticosteroid and 20 mg/week methotrexate (MTX). After one month, a partial regression of clinical and laboratory findings was observed. However, 6 months later, due to relapsed and increased complaints and findings, adalimumab 40 mg was administered. Two months later, clinical and laboratory findings apparently decreased. Senol Kobak, Hatice Yilmaz, Murat Yalcin, and Ahmet Karaarslan Copyright © 2014 Senol Kobak et al. All rights reserved. Response to Rituximab in a Case of Lupus Associated Digital Ischemia Wed, 09 Jul 2014 13:26:33 +0000 We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) and Jaccoud arthritis (JA) that sequentially developed digital ischemic lesions of the hands. In spite of follow-up treatment with glucocorticoids, immunosuppressant, antiaggregant, and potent vasodilatator agents, a serious progression to digital gangrene over a one-month period was observed. Surprisingly, her nonhealing digital lesions improved after two cycles of rituximab (RTX) administration. Orhan Küçükşahin, Nurşen Düzgün, Alexis K. Okoh, and Emre Kulahçioglu Copyright © 2014 Orhan Küçükşahin et al. All rights reserved. Treatment Resistant Severe Digital Ischemia Associated with Antiphospholipid Syndrome in a Male Patient with Systemic Sclerosis Tue, 08 Jul 2014 13:07:00 +0000 We report the case of a male patient with limited cutaneous systemic sclerosis (SSc) that was complicated with severe digital ischemia, resistant to medical treatment. Due to the lack of treatment response, further laboratory and imaging studies were conducted. Findings were compatible with antiphospholipid syndrome and oral warfarin was added to the treatment regimen. After successful anticoagulation no further recurrences of digital ischemia were seen. An underlying etiology in SSc patients with treatment resistant digital ischemic necrosis should be suspected for accompanying antiphospholipid syndrome (APS). Orhan Küçükşahin, Aşkın Ateş, Alexis K. Okoh, Emre Kulahcioglu, Murat Turgay, and Gülay Kınıklı Copyright © 2014 Orhan Küçükşahin et al. All rights reserved. Rapidly Destructive Inflammatory Arthritis of the Hip Mon, 07 Jul 2014 08:59:36 +0000 Rapidly destructive coxarthrosis (RDC) is a rare syndrome that involves aggressive hip joint destruction within 6–12 months of symptom onset with no single diagnostic laboratory, pathological, or radiographic finding. We report an original case of RDC as an initial presentation of seronegative rheumatoid arthritis (RA) in a 57-year-old Caucasian woman presenting with 6 months of progressive right groin pain and no preceding trauma or chronic steroid use. Over 5 months, she was unable to ambulate and plain films showed complete resorption of the right femoral head and erosion of the acetabulum. There were inflammatory features seen on computed tomography (CT) and magnetic resonance imaging (MRI). She required a right total hip arthroplasty, but arthritis in other joints showed improvement with triple disease modifying antirheumatic drugs (DMARD) therapy and almost complete remission with the addition of adalimumab. We contrast our case of RDC as an initial presentation of RA to 8 RDC case reports of patients with established RA. Furthermore, this case highlights the importance of obtaining serial imaging to evaluate a patient with persistent hip symptoms and rapid functional deterioration. Jenny Shu, Ian Ross, Bret Wehrli, Richard W. McCalden, and Lillian Barra Copyright © 2014 Jenny Shu et al. All rights reserved. Lung Sarcoidosis in Etanercept Treated Rheumatoid Arthritis Patient: A Case Report and Review of the Literature Thu, 03 Jul 2014 00:00:00 +0000 We report a 55-year-old female with seropositive rheumatoid arthritis for 10 years who developed large mediastinal and hilar adenopathy while receiving etanercept therapy. Chest high resolution computed tomography (HRCT) showed mediastinal lymph nodes with size of centimeters. Right paratracheal lymph node biopsy showed nonnecrotizing epithelioid granulomata. All infectious studies of pulmonary lymph node tissues were negative. Etanercept was discontinued. Follow-up HRCT 6 months later showed resolution of mediastinal lymph nodes. This report should increase awareness of pulmonary sarcoidosis development in patient treated with tumor necrosis factor-alpha blocking agent, etanercept. Supat Thongpooswan and Adriana Abrudescu Copyright © 2014 Supat Thongpooswan and Adriana Abrudescu. All rights reserved. PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature Wed, 02 Jul 2014 08:02:48 +0000 PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis. Athira Unnikrishnan, Shila Azodi, Nadeem Ansari, Megan Brown, Joshua Kamnetz, and Robert C. Uchiyama Copyright © 2014 Athira Unnikrishnan et al. All rights reserved. Tofacitinib Citrate for Ulcerative Keratitis in a Patient with Rheumatoid Arthritis Tue, 17 Jun 2014 00:00:00 +0000 Purpose. To report a case of a patient with rheumatoid arthritis (RA) treated with tofacitinib citrate. Methods. Observational case report. Results. A 59-year-old patient, with a history of rheumatoid arthritis, on methotrexate 10 mg PO qwk and IV abatacept 750 mg/month, presented with photosensitivity, foreign body sensation, pain, redness, and blurry vision of her right eye (RE). Visual acuity of the RE was 20/200 and 20/20 of the left eye (LE). The slit lamp examination of the RE revealed dryness, 2+ injection of the conjunctiva, and pericentral ulceration of the cornea with 20–30% stromal thinning, pannus, and diffuse punctate epithelial erosions. The anterior chamber appeared normal. Laboratory values revealed elevated levels of rheumatoid factor, anticyclic citrullinated peptide antibodies, and C-reactive protein. The patient was switched to tofacitinib citrate 5 mg PO b.i.d, underwent corneal gluing, and was given prednisone acetate 1% gt TID, polytrim gt TID, neomycin-polymyxin-dexameth gt QD, FreshKote lubricant 1.8% gt QID, moxifloxacin 0.5% gt QID, and preservative free artificial tears Q1H. Within one week, laboratory values normalized, symptoms diminished, and the cornea reepithelialized. Conclusion. RA can present with ulcerative keratitis. Tofacitinib citrate, steroids, and corneal gluing were found to halt the progression of keratolysis and promote reepithelialization. Philip B. Meadow, Jacqueline Nguyen, and Keerthana Kesavarapu Copyright © 2014 Philip B. Meadow et al. All rights reserved. Gouty Panniculitis with Ulcerations in a Patient with Multiple Organ Dysfunctions Sun, 15 Jun 2014 11:44:06 +0000 Gouty panniculitis is a rare manifestation of gout. Clinically, it is characterized by indurated subcutaneous nodules in nonjoint areas. Pathologically, typical characteristic gouty tophi can be seen in subcutaneous tissue. It is postulated that gouty panniculitis develops as a consequence of uric acid accumulation in the body and localized inflammatory changes in subcutaneous tissue. We report a case of a 46-year-old man with 20-year history of gout, who developed multiple subcutaneous nodules over the abdomen and right groin/thigh area over a 2-year period. After a recent episode of congestive heart failure and acute renal failure, the nodules increased in size and the overlying skin became erythematous and ulcerated. Pathologic examination demonstrated typical tophi in the dermis and subcutaneous tissue. A review of the literature yielded fifteen similar cases that had been previously reported. We conclude that gouty panniculitis may be a manifestation of undertreated gout and may be exacerbated by the deterioration of other systemic functions. Lu Wang, Crystal Rose, Paul Mellen, George Branam, and Maria M. Picken Copyright © 2014 Lu Wang et al. All rights reserved. Marked Multiple Tendinitis at the Onset of Rheumatoid Arthritis in a Patient with Heterozygous Familial Hypercholesterolemia: Ultrasonographic Observation Mon, 09 Jun 2014 12:46:08 +0000 A 59-year-old woman who had been diagnosed with heterozygous familial hypercholesterolemia developed rheumatoid arthritis (RA). She presented with marked tendinitis of the Achilles tendons, patellar tendons, and finger extensor tendons at the onset of RA. Ultrasonographic examination revealed that tendon lesions were predominantly tendinitis rather than paratenonitis, and that the tendinitis was of the noninsertional variety, rather than the insertional variety. Preexisting tendon xanthomas might have contributed to the unusually dominant noninsertional tendinitis of multiple tendons. Takeshi Suzuki and Akiko Okamoto Copyright © 2014 Takeshi Suzuki and Akiko Okamoto. All rights reserved. Methotrexate Induced Pancytopenia Tue, 27 May 2014 13:31:12 +0000 The well-reported methotrexate (MTX) toxicities are based on the duration and cumulative dosing of drug. The typical toxicities can be predicted by the timing of drug administration, where mucositis occurs as an earlier effect, while myelosuppression and the sequelae of pancytopenia occur later after MTX administration. Despite these well-known toxicities, low dose MTX therapy can become problematic, in particular with the elderly, who are at a greater risk for significant myelosuppression. We present a case of a 73-year-old female with pancytopenia causing severe neutropenia, mucocutaneous bleeding, and bruising and requiring intravenous antibiotic therapy and limited transfusion dependence as a result of low dose daily MTX for rheumatoid arthritis. Fernando Gonzalez-Ibarra, Sahar Eivaz-Mohammadi, Shiri Surapaneni, Hazem Alsaadi, Amer K. Syed, Simon Badin, Valentin Marian, and Mazhar Elamir Copyright © 2014 Fernando Gonzalez-Ibarra et al. All rights reserved. Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis Sun, 18 May 2014 12:04:15 +0000 Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions. Basheer Tashtoush, Roya Memarpour, Yasmin Johnston, and Jose Ramirez Copyright © 2014 Basheer Tashtoush et al. All rights reserved. Possible Association of Etanercept, Venous Thrombosis, and Induction of Antiphospholipid Syndrome Thu, 15 May 2014 08:54:00 +0000 Tumor necrosis factor α (TNF α) inhibitors are commonly used for treatment of aggressive rheumatoid arthritis and other rheumatic diseases. Etanercept is one of the medications approved for treatment of rheumatoid arthritis. Though many studies have documented the safety and efficacy of these medications, evidence for adverse effects is emerging including cancer, infections, and cardiovascular disease. There have been studies showing that these medications induce autoantibody production, including antinuclear antibodies and anti-dsDNA antibodies. Limited data exists, however, regarding induction of antiphospholipid antibodies (APLs) by TNF α inhibitors, including anticardiolipin antibodies (ACLs), lupus anticoagulant (LAC), and anti-β2-glycoprotein I (anti-β2 GPI), or an association between antibody development and clinical manifestations. In this case series, we describe five patients who developed venous thromboembolism (VTE) and APLs while receiving etanercept therapy. All five of our patients met the criteria for diagnosis of APS after receiving etanercept. Our case series supports the association between etanercept, APLs, and VTE. We believe that testing for APLs prior to initiation of anti-TNF therapy is reasonable, given this relationship and the risks associated with VTE. Shanti Virupannavar, Anthony Brandau, Carla Guggenheim, and Heather Laird-Fick Copyright © 2014 Shanti Virupannavar et al. All rights reserved. A Case of Blau Syndrome Sun, 04 May 2014 00:00:00 +0000 We present a case of systemic granulomatous disorder/Blau syndrome. A patient was seen at our clinic with a diagnosis of Juvenile Idiopathic Arthritis (JIA). He was diagnosed with polyarticular JIA when he was two years old, at that time primary manifestations included inflammation of the hand and wrist joints bilaterally, later he developed ocular symptoms, which were attributed to JIA. He had liver, skin, pulmonary manifestations, and diagnostic workup including biopsy revealed granulomatous inflammation of these sites. During the diagnostic workup, he had worsening of ocular complaints, retinal exam showed panuveitis with multifocal choroiditis. These ocular findings are not seen in JIA, this, along with his other systemic manifestations, led us to revisit the diagnosis. Laboratory testing for genetic mutation for Blau syndrome was done and came back positive. Now all of his systemic findings were placed under one umbrella of systemic granulomatous syndrome/Blau syndrome. Due to worsening of ocular manifestations, he was started on Adalimumab with marked improvement of ocular and systemic manifestations and is followed by team that consists of Rheumatologist, Ophthalmologist, and Gastroenterologist. Krati Chauhan and Clement Michet Copyright © 2014 Krati Chauhan and Clement Michet. All rights reserved. Occurrence of Psoriatic Arthritis during Interferon Beta 1a Treatment for Multiple Sclerosis Tue, 15 Apr 2014 14:07:38 +0000 Interferon beta (IFN-β) is the first line therapy of relapsing-remitting multiple sclerosis. IFN-β is a cytokine that can contribute to the development of systemic autoimmune disease including psoriasis. The development or the exacerbation of psoriasis during IFN-β treatment has been previously observed. We report the occurrence of arthritis and dactylitis in a multiple sclerosis patient with preexisting psoriasis diagnosed as a psoriatic arthritis. The IL-23/Th17 pathway is involved in psoriasis and psoriatic arthritis and it has been suggested that IFN-β therapy in patients with Th17-mediated disease may be detrimental. Together with previous similar reports, our case suggests that IFN-β should certainly be used with caution in patients with concomitant systemic autoimmune disease with IL-23/Th17 involvement. Éric Toussirot, Matthieu Béreau, Marie Bossert, Imad Malkoun, and Anne Lohse Copyright © 2014 Éric Toussirot et al. All rights reserved. A Case of Rheumatoid Arthritis and Limited Systemic Sclerosis Overlap Successfully Treated with Tocilizumab for Arthritis and Concomitant Generalized Lymphadenopathy and Primary Biliary Cirrhosis Tue, 15 Apr 2014 07:19:57 +0000 A 57-year-old woman with rheumatoid arthritis (RA) and limited systemic sclerosis (lSSc) was suspected to have lymphadenopathy and primary biliary cirrhosis (PBC). Lymph node biopsy showed reactive follicular lymphadenopathy with intrafollicular plasmacyte infiltration that was interleukin-6 positive by immunohistostaining. Because of gradually worsening arthritis, tocilizumab was administered and arthritis improved markedly. Interestingly, lymphadenopathy and PBC improved simultaneously. This suggested that interleukin-6 might play an important role in reactive lymphadenopathy and PBC associated with RA/lSSc. Eiko Saito, Shinji Sato, Shinichi Nogi, Noriko Sasaki, Naofumi Chinen, Kiri Honda, Takayuki Wakabayashi, Chiho Yamada, Naoya Nakamura, and Yasuo Suzuki Copyright © 2014 Eiko Saito et al. All rights reserved. Myelofibrosis-Related Arthritis Successfully Treated with Hydroxyurea Thu, 10 Apr 2014 12:37:43 +0000 A 62-year-old woman suffering from one-year lasting, nonerosive peripheral arthritides with general health impairment and high acute-phase reactant levels was admitted to rheumatology department. The patient had suffered from chronic polyarthralgia and a thrombocytosis had been discovered 9 years before, with a recent increase in platelet count. All immunological blood tests were negative. Corticosteroid and methotrexate treatments improved pain, swollen joint count, and systemic inflammation. However, joints remained stiff and painful with two swollen wrists and persistent thrombocytosis. An iliac bone marrow biopsy was performed, showing primary myelofibrosis. Hydroxyurea treatment (500 mg per day) allowed to achieve complete and prolonged clinical and biological remission. After 6 months, a new disease flare occurred. The patient reached remission again after hydroxyurea dose increased to 1500 mg per day. This supports the hypothesis of idiopathic myelofibrosis-associated seronegative polyarthritis. This is the first reported case in which haemopathy-targeted treatment using hydroxyurea induced arthritis remission. Xavier Guillot, Marius Moldovan, Claire Vidon, and Daniel Wendling Copyright © 2014 Xavier Guillot et al. All rights reserved. A Debilitating Orthopaedic Complication following Corticosteroid Therapy for Polymyalgia Rheumatica Sun, 06 Apr 2014 12:33:47 +0000 Avascular necrosis (AVN) of the scaphoid secondary to corticosteroid use is a rare entity. Previous reports in the literature refer to chronic steroid intake. We report a case secondary to low dose, short term use. AVN has a multifactorial cellular and genetic aetiology and most frequently affects the femoral head. Diagnosis relies on a high index of suspicion and early magnetic resonance (MR) scanning. Treatment options are similar to those of traumatic scaphoid nonunions and include vascularised bone grafting and scaphoid excision. Polymyalgia Rheumatica is a common condition and its treatment is led by corticosteroid use. Mild to moderate strengths are advocated. However in our report we show that even with small doses serious adverse effects can be encountered. Paul Rai and Vinay Takwale Copyright © 2014 Paul Rai and Vinay Takwale. All rights reserved. Amyloidoma, an Unusual Cause of Fracture Sun, 16 Mar 2014 07:22:49 +0000 We report a case of a spontaneous hip fracture in a context of dysglobulinemia. The bone histologic examination found amyloidoma. Amyloidoma is an overload pathology and an unusual cause of fracture. In most of the cases, it is associated with myeloma and the difference between bone invasion of myeloma and amyloidoma in an osteolytic radiographic picture is not easy but is of importance because prognosis and treatment may be totally different. Thus, in the context of dysglobulinemia, one must keep in mind that spontaneous bone fracture may be due to amyloidoma with another prognosis. Frank Verhoeven, Clément Prati, and Daniel Wendling Copyright © 2014 Frank Verhoeven et al. All rights reserved.