Objective. To validate the 2011 modification of the 2010 American College of Rheumatology (ACR) preliminary criteria for the diagnosis of fibromyalgia (2011ModCr) and develop alternative criteria in a sample of patients with diverse pain disorders that are commonly seen in everyday practice by pain specialists, rheumatologists, and psychologists. Methods. Eight clinicians from geographically varied locations in the US evaluated patients with chronic pain and psychiatric disorders using a standard set of questions that included the 2011ModCr questions, the Symptom Impact Questionnaire (SIQR), a 28-area pain location inventory (PLI), and the Short Form 36. Alternative diagnostic criteria were developed from the same data set using logistic regression and receiver operating curve analysis. Results. Complete data on 321 patients were evaluated; there were 135 patients with fibromyalgia (according to the 1990 ACR criteria) and 186 patients with 16 other common chronic pain problems. Comparing the 2011ModCr with the 1990 ACR criteria provided a sensitivity of 83%, a specificity of 67%, and a correct classification of 74%. Alternative criteria were derived from the 10-item symptom score from the SIQR symptoms and the 28-area PLI. Maximal diagnostic accuracy was obtained with >17 pain sites (range 0 -28) and an SIQR symptom score of >21 (range 0 -50). These alternative criteria had a diagnostic sensitivity of 81%, a specificity of 80%, and a correct classification of 80%. Conclusion. The 2011ModCr had robust operating characteristics. Alternative criteria based on symptom items from the SIQR and pain locations from the PLI had comparable operating characteristics, with somewhat better specificity and ease of use.

No abstract

Original ResearchTo describe clinical features and outcomes of seven patients with pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma in the setting of underlying primary Sjögren's syndrome from a single center, we reviewed medical records of consecutive patients with pulmonary MALT lymphoma evaluated at our facility from January 1, 1999 to December 31, 2015 for clinical features, laboratory, pathologic and radiographic findings, management, and outcomes. Out of 13 patients with pulmonary MALT lymphoma, 7 (54 %) met the criteria for Sjögren's syndrome. The mean age at lymphoma diagnosis was 66 years; male-female ratio was 1:6. One-third of patients were asymptomatic at the time lymphoma was discovered. When symptomatic, patients reported nonspecific pulmonary complaints such as cough and dyspnea. All patients had positive antinuclear antibody and anti-SSA/Ro antibody. Rheumatoid factor was positive in six cases. A monoclonal gammopathy was present in three patients; the remaining four had polyclonal hypergammaglobulinemia. The radiologic, morphologic, and immunohistochemical features of primary Sjögren's syndrome-associated pulmonary MALT lymphomas did not differ significantly from pulmonary MALT lymphoma cases in general. All treatment modalities used resulted in complete and sustained response. One patient died 11 years after initial diagnosis with no lymphoma but of another cause. The remaining six patients are still alive and disease-free to date. The present series confirms the favorable course of pulmonary MALT lymphoma in Sjögren's patients. The overall imaging and pathologic features are in accordance with pulmonary MALT lymphoma not associated with primary Sjögren's syndrome. Further studies should be carried out in order to better understand pulmonary MALT lymphomagenesis, treatment, and outcomes in Sjögren's patients.

Bone and bone marrow involvement in sarcoidosis have been infrequently reported. We aimed to describe the clinical features, radiological descriptions, pathological examinations, and outcomes of three patients with osseous sarcoidosis and one patient with bone marrow sarcoidosis seen at our institution. Our case series included fluorodeoxyglucose positron emission tomography descriptions in assessing the whole-body extent of sarcoidosis. In the era of advanced imaging, large bone and axial skeleton sarcoidosis lesions are more common than previously reported.

To describe clinical features and outcomes of 26 patients with idiopathic retroperitoneal fibrosis from a single center, we reviewed medical records of consecutive patients with idiopathic retroperitoneal fibrosis evaluated at our facility from January 1, 1998 to December 31, 2013 for clinical features, laboratory and radiographic findings, management, and outcomes. Twenty-six patients met criteria for idiopathic retroperitoneal fibrosis and were included in the study. Median age at diagnosis was 58 years; male-female ratio was 3.3:1.0. Median duration of symptoms was 7 weeks. Abdominal, flank, and/or low back pain were the most common presenting symptoms. Four patients (15 %) had associated autoimmune or fibrosing disorders. Baseline erythrocyte sedimentation rate was elevated in 17 (77 %) of 22 patients tested and C-reactive protein was elevated in 10 (56 %) of 18 patients tested. Hydronephrosis was present in 17 (68 %) patients; 8 (47 %) of 17 had bilateral hydronephrosis. Retroperitoneal mass biopsy was performed in 18 (69 %) patients. Two patients had idiopathic retroperitoneal fibrosis classifiable as IgG4-related disease. Therapy consisted of medications alone in 7 cases, surgical interventions alone in 7 cases, and a combination in 11 cases. One patient achieved remission with no treatment. Most patients treated medically received initial corticosteroids. Methotrexate (1 case), azathioprine (1 case), mycophenolate mofetil (1 case), and tamoxifen (5 cases) were used. No relapses occurred after a median 5-year follow-up. Two (8 %) patients died; five (19 %) developed cancer after diagnosis. In this series, we emphasize the importance of early diagnosis and therapy for overall favorable prognosis of idiopathic retroperitoneal fibrosis.

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The Stevens-Johnson syndrome (SJS) classically involves a targetoid skin rash and the association of the oral mucosa, genitals, and conjunctivae. Recently, there have been several documentations of an incomplete presentation of this syndrome, without the typical rash, usually associated with the mycoplasma pneumoniae infection. Our case illustrates that this important clinical diagnosis should not be missed due to its atypical presentation.

Erythema multiforme (EM) and systemic lupus erythematosus (SLE) are common diseases. Their coexistence is known as Rowell syndrome (RS), first described in 1963. Only few cases of RS have been described and some of them questioned its existence. We present two cases of SLE in the setting of a newly developed EM-like eruption, which shares many similarities with the so-called Rowell syndrome.