Case Reports in Rheumatology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. High-Dose Subcutaneous Immunoglobulins for the Treatment of Severe Treatment-Resistant Polymyositis Wed, 16 Jul 2014 12:07:35 +0000 http://www.hindawi.com/journals/crirh/2014/458231/ 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 http://www.hindawi.com/journals/crirh/2014/416825/ 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 http://www.hindawi.com/journals/crirh/2014/763608/ 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 http://www.hindawi.com/journals/crirh/2014/291382/ 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 http://www.hindawi.com/journals/crirh/2014/160252/ 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 http://www.hindawi.com/journals/crirh/2014/358567/ 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 http://www.hindawi.com/journals/crirh/2014/582094/ 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 http://www.hindawi.com/journals/crirh/2014/403452/ 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 http://www.hindawi.com/journals/crirh/2014/320940/ 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 http://www.hindawi.com/journals/crirh/2014/486348/ 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 http://www.hindawi.com/journals/crirh/2014/679580/ 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 http://www.hindawi.com/journals/crirh/2014/850364/ 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 http://www.hindawi.com/journals/crirh/2014/801072/ 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 http://www.hindawi.com/journals/crirh/2014/216056/ 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 http://www.hindawi.com/journals/crirh/2014/949317/ 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 http://www.hindawi.com/journals/crirh/2014/386328/ 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 http://www.hindawi.com/journals/crirh/2014/869743/ 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 http://www.hindawi.com/journals/crirh/2014/515361/ 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 http://www.hindawi.com/journals/crirh/2014/424056/ 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. Coexistence of Ankylosing Spondylitis and Löfgren’s Syndrome Tue, 11 Mar 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/747698/ A 46-year-old male patient diagnosed with ankylosing spondylitis presented to our polyclinic with complaints of pain, swelling, and limitation in joint mobility in both ankles and erythema nodosum skin lesions in both pretibial sites. The sacroiliac joint graphy and the MRI taken revealed active and chronic sacroiliitis. On the thorax CT, multiple mediastinal and hilar lymphadenopathies were reported. Mediastinoscopic excisional lymph node biopsy was taken and noncalcified granulomatous structures, lymphocytes, and histiocytes were determined on histopathological examination. The patients were diagnosed with ankylosing spondylitis, sarcoidosis, and Löfgren’s syndrome. NSAIDs, sulfasalazine, and low dose corticosteroid were started. Significant regression was seen in the patient’s subjective and laboratory assessments. Senol Kobak, Fidan Sever, Oya Sivrikoz, and Ahmet Karaarslan Copyright © 2014 Senol Kobak et al. All rights reserved. Developing of Granulomatosis with Polyangiitis during Etanercept Therapy Thu, 06 Mar 2014 12:11:32 +0000 http://www.hindawi.com/journals/crirh/2014/210108/ We describe a 67-year-old woman who developed c-ANCA positive vasculitis with involvement in eyes, skin, kidney, peripheral nerves, and upper and lower airway during treatment with etanercept therapy for rheumatoid arthritis. A diagnosis of Granulomatosis with Polyangiitis was done. Thus, anti-TNF therapy may be associated with the development of ANCA positive vasculitis. María Clara Ortiz-Sierra, Andrés Felipe Echeverri, Gabriel J. Tobón, and Carlos Alberto Cañas Copyright © 2014 María Clara Ortiz-Sierra et al. All rights reserved. An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation Tue, 04 Mar 2014 07:27:47 +0000 http://www.hindawi.com/journals/crirh/2014/128623/ Case. A 34-year-old African-American female with a history of adult-onset Still’s disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizure en route via ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital’s burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired. Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD. Rajaie Namas, Naveen Nannapaneni, Malini Venkatram, Gulcin Altinok, Miriam Levine, and J. Patricia Dhar Copyright © 2014 Rajaie Namas et al. All rights reserved. Ramsay Hunt Syndrome in a Patient with Rheumatoid Arthritis after Treatment with Infliximab Sun, 09 Feb 2014 07:04:03 +0000 http://www.hindawi.com/journals/crirh/2014/897647/ A 39-year-old female patient with rheumatoid arthritis developed Ramsay Hunt syndrome after infliximab treatment. This condition is caused by the reactivation of varicella zoster virus infection in the geniculate ganglion of facial nerve in the host’s immunosuppression. She was treated immediately with valaciclovir and hydrocortisone, and the complete recovery was achieved at 6 months after the onset. This is the first report of Ramsay Hunt syndrome as an adverse effect of infliximab in rheumatoid arthritis. Yoshio Nagayama, Naoki Matsushiro, Akihide Nampei, Hideo Hashimoto, and Kenrin Shi Copyright © 2014 Yoshio Nagayama et al. All rights reserved. Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis Wed, 05 Feb 2014 08:09:40 +0000 http://www.hindawi.com/journals/crirh/2014/653837/ A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. Senol Kobak, Hatice Yilmaz, Ahmet Karaarslan, and Murat Yalcin Copyright © 2014 Senol Kobak et al. All rights reserved. Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis Sun, 19 Jan 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/549641/ Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant. Hakan Emmungil, Melike Kalfa, Burcu Başarık, Hasip Kahraman, Ferhat Tanhan, Banu Yaman, Atiye Öztürk, Zehra Erdemir, Gülşen Kandiloğlu, Vedat İnal, and Yasemin Kabasakal Copyright © 2014 Hakan Emmungil et al. All rights reserved. Milwaukee Shoulder Syndrome Thu, 16 Jan 2014 11:07:31 +0000 http://www.hindawi.com/journals/crirh/2014/458708/ Milwaukee shoulder syndrome (MSS) is a rare destructive, calcium phosphate crystalline arthropathy. It encompasses an effusion that is noninflammatory with numerous aggregates of calcium hydroxyapatite crystals in the synovial fluid, associated with rotator cuff defects. We describe a patient that presented with recurrent shoulder pain and swelling with characteristic radiographic changes and MSS was confirmed on aspiration of the synovial fluid. Channa Vasanth Nadarajah and Immo Weichert Copyright © 2014 Channa Vasanth Nadarajah and Immo Weichert. All rights reserved. Mycobacterium haemophilum as the Initial Presentation of a B-Cell Lymphoma in a Liver Transplant Patient Sun, 12 Jan 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/742978/ A 66-year-old woman presented with pustular lesions of her face, trunk, and limbs and an acute arthritis of the knees and elbows. She had a complex medical background and had been on immunosuppressants for three years after a liver transplant. Tissue samples from her skin lesions and synovial fluid showed acid-fast bacilli. Mycobacterium haemophilum, an atypical mycobacteria, was later grown on culture. During her treatment with combination antibiotic therapy, she developed a pronounced generalised lymphadenopathy. Histology showed features of a diffuse B-cell lymphoma, a posttransplant lymphoproliferative disorder (PTLD). T. Doherty, M. Lynn, A. Cavazza, E. Sames, and R. Hughes Copyright © 2014 T. Doherty et al. All rights reserved. Diagnosis and Assessment of Disease Activity in Takayasu Arteritis: A Childhood Case Illustrating the Challenge Sun, 05 Jan 2014 14:18:59 +0000 http://www.hindawi.com/journals/crirh/2014/603171/ Takayasu Arteritis (TA) is a rare, debilitating large vessel vasculitis occurring in patients of all ages, including infants, but the disease most commonly presents in the third decade. Diagnosis is often delayed and consequently TA is associated with significant morbidity and mortality. Accurate methods of monitoring disease activity or damage are lacking and currently rely on a combination of clinical features, blood inflammatory markers, and imaging modalities. In this report we describe a case of a 14-year-old boy with childhood-onset TA who, despite extensive negative investigations, did indeed have on-going active large vessel vasculitis with fatal outcome. Postmortem analysis demonstrated more extensive and active disease than originally identified. This report illustrates and discusses the limitations of current modalities for the detection and monitoring of disease activity and damage in large vessel vasculitis. Clinicians must be aware of these limitations and challenges if we are to strive for better outcomes in TA. L. Watson, P. Brogan, I. Peart, C. Landes, N. Barnes, and G. Cleary Copyright © 2014 L. Watson et al. All rights reserved. A Case of Granulomatosis with Polyangiitis Causing Hydroureter and Hydronephrosis Thu, 02 Jan 2014 10:04:37 +0000 http://www.hindawi.com/journals/crirh/2014/713957/ Granulomatosis with Polyangiitis (GPA, formerly known as Wegener) is a systemic vasculitis characterized by granulomatous involving upper and lower respiratory tract and can also cause necrotizing glomerulonephritis (Umemoto et al. 2012 and Takala et al. 2011). GPA is associated with antineutrophil cytoplasmic autoantibodies (ANCA) against serine proteinase 3 (PR3) (Takala et al. 2011, Dufour et al. 2012, and Berthoux et al. 2011). This disease usually starts with involvement of the upper and lower respiratory tracts and also can involve kidney, eyes, skin, central and peripheral nervous systems, and gastrointestinal tract (Umemoto et al. 2012, Takala et al. 2011, and Berthoux et al. 2011). We describe a case of GPA that presented with abdominal pain. Computed tomography (CT) scan with contrast showed right sided moderate hydronephrosis and hydroureter, to the level of the right common iliac artery. There was also mural segmental thickening in common iliac artery which was thought to be the cause of the ureteral obstruction and hydronephrosis. Our case shows that mural segmental thickening in common iliac artery happened due to GPA and caused hydronephrosis. In addition, most of the cases with hydronephrosis due to GPA went through urology intervention such as stent placement but in our case hydronephrosis resolved with medical management. Farzin Farpour and Adriana Abrudescu Copyright © 2014 Farzin Farpour and Adriana Abrudescu. All rights reserved. A Rare Cause of Pericardial Effusion: Giant Cell Arteritis Thu, 02 Jan 2014 09:17:55 +0000 http://www.hindawi.com/journals/crirh/2014/424295/ Giant cell arteritis is a granulomatous vasculitis characterized by medium or large sized vessel involvement. Although extracranial branches of the carotid artery are typically involved, involvement of aorta and its major branches can also be seen. Cardiac involvement has been encountered less frequently and pericardial effusion is rarely encountered. In this paper, a case has been presented in which pericardial effusion was determined during the examination and diagnosis was giant cell arteritis. Turker Tasliyurt, Hakan Sivgin, Lutfu Bekar, Safak Sahin, Suheyla Uzun Kaya, Resit Dogan Koseoglu, Faruk Kutluturk, and Abdulkerim Yilmaz Copyright © 2014 Turker Tasliyurt et al. All rights reserved.