Case Reports in Rheumatology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . 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. Ascites as the Presenting Symptom of Multiple Myeloma in a Scleroderma Patient Thu, 20 Nov 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/235958/ Several reports have demonstrated associations between scleroderma and cancer and between multiple myeloma and autoimmune diseases. Few papers have also reported the concurrence of scleroderma and multiple myeloma. We report a case of multiple myeloma that developed in a male patient after 28 years of fulfilling a diagnosis of scleroderma. The main presenting feature of multiple myeloma was ascites, solely explained by the increased vascular permeability that occurs in this disease. Nadeen Hilal and Adnan Atallah Copyright © 2014 Nadeen Hilal and Adnan Atallah. All rights reserved. A Rare Case of Neuromyelitis Optica Spectrum Disorder in Patient with Sjogren’s Syndrome Wed, 19 Nov 2014 06:56:42 +0000 http://www.hindawi.com/journals/crirh/2014/158165/ We report a 48-year-old female with the history of Sjogren’s syndrome who presented with 3-week history of tingling, numbness, and shooting back, waist, and bilateral leg pain and numbness in the pelvic region with urinary and bowel incontinence. Physical examination was remarkable for reduced motor power in both lower extremities with spasticity. Sensory deficit was noted at the T6 level. Laboratory investigation revealed elevated ESR and CRP and positive serum antiaquaporin-4 IgG. Thoracic and lumbar magnetic resonance imaging revealed abnormal patchy areas, leptomeningeal enhancement through the thoracic cord extending from T3 through T6 levels, without evidence of cord compression. Impression of neuromyelitis optica spectrum disorder was made and patient was treated with methylprednisolone intravenously followed by tapering oral prednisone. Neurological symptoms gradually improved with resolution of bowel and urinary incontinence. In a patient with Sjogren’s syndrome who presents with neurological complaints, the possibility of neuromyelitis optica or neuromyelitis optica spectrum disorder should be considered. Awareness of the possibility of CNS disease is important due to the serious nature of CNS complications, some of which are treatable with immunosuppressants. Our patient with Sjogren’s syndrome who presented with myelopathy benefited from early recognition and institution of appropriate therapy. Supat Thongpooswan, Bikash Chapagain, and Sabiha Bandagi Copyright © 2014 Supat Thongpooswan et al. All rights reserved. A Case of Polyarteritis Nodosa Associated with Cytomegalovirus Infection Wed, 12 Nov 2014 09:07:48 +0000 http://www.hindawi.com/journals/crirh/2014/604874/ A 77-year-old man suffering from prolonged fever of unknown origin and bilateral leg edema was referred to our hospital. On physical examination, he had fever, general fatigue, bilateral lower leg edema, and muscle weakness of the right upper extremity and left lower extremity. Neurological examination indicated motor and sensory disturbance. Electromyography revealed mononeuritis multiplex and myopathy. A biopsy of the left biceps muscle indicated necrotizing vasculitis with fibrinoid necrosis. Considering all the data together, he was diagnosed as having polyarteritis nodosa (PAN) and concurrent active cytomegalovirus (CMV) infection. His symptoms improved promptly on treatment with 50 mg of prednisolone. This case emphasizes the importance of CMV infection as one of possible etiologies of PAN and reports a therapeutic strategy for this syndrome. Maiko Kouchi, Shinji Sato, Masahiro Kamono, Akiko Taoda, Kazuyuki Iijima, Atsushi Mizuma, Ruriko Kitao, Masatoshi Mihara, Hideki Ozawa, Tadayuki Ishihara, Atsushi Takagi, and Yasuo Suzuki Copyright © 2014 Maiko Kouchi et al. All rights reserved. Effectiveness and Safety of Infliximab in Two Cases of Severe Chondrocalcinosis: Nine Years of Follow-Up Tue, 11 Nov 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/536856/ Objectives. To investigate the efficacy of infliximab in the treatment of severe calcium pyrophosphate deposition diseases (CPPD). Methods. Two patients with severe CPPD and diffuse idiopathic skeletal hyperostosis- (DISH-) like phenotype are described. Both patients were resistant to therapy with nonsteroidal anti-inflammatory drugs (NSAIDs). Both patients were treated with infliximab, a TNF-α receptor antagonist, for nine years. Results. Treatment with infliximab resulted in major clinical and laboratory improvements without relevant side effects. Conclusions. These results suggest that infliximab may be an effective treatment of severe CPDD. Jácome Bruges-Armas, Bruno F. Bettencourt, Ana R. Couto, Manuela Lima, Ana M. Rodrigues, Nathan Vastesaeger, and Matthew A. Brown Copyright © 2014 Jácome Bruges-Armas et al. All rights reserved. Pulmonary Nodules as an Initial Manifestation of Behçet’s Disease Sun, 09 Nov 2014 09:14:53 +0000 http://www.hindawi.com/journals/crirh/2014/869817/ Behçet’s disease (BD) is a systemic vasculopathy, characterized by recurrent oral aphthae, genital ulcers, uveitis, and skin lesions. Although vascular involvement, including venous and arteries of any size, is a usual manifestation, cases with pulmonary thrombosis as the initial symptom are not common in the absence of pulmonary artery aneurysm (PAA). This report describes a 36-year-old man with recurrent fever, nonmassive hemoptysis, and persistent cough with lung nodules in CT scan who had undergone open lung biopsy. On the basis of morphological findings, BD was suggested and more precise evaluation confirmed the diagnosis. M. Malekmohammad and A. Emamifar Copyright © 2014 M. Malekmohammad and A. Emamifar. All rights reserved. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease Sun, 09 Nov 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/195617/ Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM) from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis. Shinji Sato, Shinichi Nogi, Noriko Sasaki, Naofumi Chinen, Kiri Honda, Eiko Saito, Takayuki Wakabayashi, Chiho Yamada, and Yasuo Suzuki Copyright © 2014 Shinji Sato et al. All rights reserved. Acute Onset Polymyositis after Prolactinoma Extirpation Thu, 06 Nov 2014 13:39:20 +0000 http://www.hindawi.com/journals/crirh/2014/329059/ Hyperprolactinemia has been related to autoimmune diseases. Herein, we describe a case of a female with a prolactin producer pituitary macroadenoma who developed severe polymyositis one month after its removal. The patient had very high levels of CPK and muscle biopsy showed remarkable inflammatory infiltration. Steroid therapy was followed with total recovery. To the best of our knowledge, this is the first case reported of acute polymyositis after pituitary macroadenoma exeresis. Juan Jakez-Ocampo, Yemil Atisha-Fregoso, and Luis Llorente Copyright © 2014 Juan Jakez-Ocampo et al. All rights reserved. Leprosy Mimicking Common Rheumatologic Entities: A Trial for the Clinician in the Era of Biologics Thu, 06 Nov 2014 00:00:00 +0000 http://www.hindawi.com/journals/crirh/2014/429698/ Rheumatoid arthritis and seronegative spondyloarthritis, which make up the lion’s share of cases attending a rheumatology clinic, are relatively easy to diagnose. However, when an entity of infective aetiology like leprosy known to be a great mimic of different autoimmune conditions presents with features similar to these, the possibility of it being diagnosed at the outset is very slim indeed. The ease with which the diagnosis of leprosy can be missed assumes sinister proportions as the use of disease modifying agents can have deleterious effects in these patients. In the era of increasing availability and use of biologic disease modifying agents, it is imperative not only to actively rule out the presence of leprosy but also to make it a part of the prebiologic screening of patients in whom biologics are being planned to be administered, especially in leprosy endemic areas. Deepak Rath, Shrinath Bhargava, and Bijit Kumar Kundu Copyright © 2014 Deepak Rath et al. All rights reserved. Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome Tue, 21 Oct 2014 06:23:06 +0000 http://www.hindawi.com/journals/crirh/2014/491937/ Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities. Zeinab A. El-Sayed, Hanaa M. El-Awady, Zeinab E. Hassan, Tamer M. H. Adham, Hossam M. Mostafa, and Nadia G. Elhefnawy Copyright © 2014 Zeinab A. El-Sayed et al. All rights reserved. Acute Hemorrhagic Myositis in Inflammatory Myopathy and Review of the Literature Tue, 14 Oct 2014 12:49:38 +0000 http://www.hindawi.com/journals/crirh/2014/639756/ 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 http://www.hindawi.com/journals/crirh/2014/809629/ 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 http://www.hindawi.com/journals/crirh/2014/893836/ 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 http://www.hindawi.com/journals/crirh/2014/768321/ 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 http://www.hindawi.com/journals/crirh/2014/835050/ 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 http://www.hindawi.com/journals/crirh/2014/359605/ 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 http://www.hindawi.com/journals/crirh/2014/353782/ 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 http://www.hindawi.com/journals/crirh/2014/341304/ 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 http://www.hindawi.com/journals/crirh/2014/873189/ 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 http://www.hindawi.com/journals/crirh/2014/925961/ 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 http://www.hindawi.com/journals/crirh/2014/246852/ 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 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.