Sjögren Syndrome Associated with Inflammatory Muscle Diseases.

OBJECTIVES: Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function that may affect multiple organ systems. The association of SS with inflammatory myopathies (IM), a group of diseases characterized by chronic inflammation of striated muscle and skin has been infrequently described. METHODS: We present two cases diagnosed with SS who developed IM. We have also conducted a review of the English literature to depict all available clinical evidence on the clinical association of SS with IM. RESULTS: Two female patients diagnosed with SS developed polymyositis (PM) and inclusion body myositis (IBM) respectively. The literature review identified 24 cases with coexistence of the two autoimmune conditions (SS and IM). Twenty-two patients were females and two males. Eight patients were diagnosed with IBM, 15 were diagnosed with PM and 1 with dermatomyositis. All patients had biopsy proven IM. CONCLUSIONS: There is evidence of clinical association of primary SS and IM especially with IBM and PM. Patients with SS and symptoms of muscle weakness should be investigated for associated IM.

International consensus: What else can we do to improve diagnosis and therapeutic strategies in patients affected by autoimmune rheumatic diseases (rheumatoid arthritis, spondyloarthritides, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome and Sjogren's syndrome)?: The unmet needs and the clinical grey zone in autoimmune disease management.

Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians. After a systematic review of the literature, the results and the discussions from each working group were summarised in different statements. Due to the differences among the diseases and their heterogeneity, a large number of statements was produced and voted by the Experts to reach a consensus in a plenary session. At all the steps of this process, including the initial discussions by the steering committee, the identification of the unmet needs, the expansion of the working group and finally the development of statements, a large agreement was attained. This work confirmed that several unmet needs may be identified and despite the development of new therapeutic strategies as well as a better understanding of the effects of existing therapies, many open questions still remain in this field, suggesting a research agenda for the future and specific clinical suggestions which may allow physicians to better manage those clinical conditions still lacking of scientific clarity.

Predictors of renal histopathology in antineutrophil cytoplasmic antibody associated glomerulonephritis.

OBJECTIVES: Prompt, aggressive therapy is vital for anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. In this regard, we aimed to identify predictors of distinct renal histopathological classes at the time of clinical diagnosis. PATIENTS & METHODS: An inception cohort of patients with biopsy proven ANCA-associated glomerulonephritis was studied retrospectively. Demographics, clinical, laboratory, serological and radiological parameters were analyzed. Patients were classified on the basis of renal histopathology. A risk score was developed for each histopathological class using univariate and stepwise logistic regression analyses. RESULTS: Variables independently associated with focal class included disease duration up to diagnosis <8 weeks, absence of erythrocyte casts by urine microscopy and eGFR >49 ml/min/1.73 m(2); with crescentic class >40 erythrocytes/hpf, identification of erythrocyte casts in urine, upper respiratory tract involvement and eGFR <49 ml/min/1.73 m(2); with mixed class age >54 years, male gender, and absence of upper respiratory tract involvement. In the presence of these risk factors a predictive risk score for each histopathological classes was calculated: odds ratio, 95% confidence intervals (CI), for focal class (≥2 risk factors, 20.8 (95% CI: 5.1-84.2), p < 0.0001, and 441.0 (95% CI: 16.8-11,590), p = 0.0003 for crescentic class (≥3 risk factors) while the small number of patients in the mixed and sclerotic class precluded any estimates. CONCLUSION: We propose a predictive algorithm of specific histolopathological classes of ANCA-associated glomerulonephritis, which might provide a crude estimation of the disease activity in the glomeruli at presentation. This tool might assist the clinician in making decisions regarding the level of intensity of inductive immunosuppressive therapy at clinical diagnosis.

Tuberculous pyomyositis in a rheumatoid arthritis patient treated with anakinra.

Musculoskeletal tuberculosis (TB) occurs in only 3% of patients with TB while tuberculous pyomyositis is rare. It usually affects immunocompromised or patients with underlying comorbidities. We present a case of tuberculous pyomyositis in a 85-year-old Caucasian patient with rheumatoid arthritis (RA) treated with steroids and anakinra. The patient presented with fever as well as redness, swelling and induration on the lateral side of the hip and thigh. Under ultrasound guidance fluid collection of the thigh was aspirated. Polymerase chain reaction (PCR) and cultures of the fluid were positive for Mycobacterium TB. The patient underwent bronchoscopy. PCR and cultures from the bronchoalveolar lavage were also positive for Mycobacterium TB. The patient was treated with anti TB treatment with amelioration of the inflammation in the hip and thigh. This is the first reported case of tuberculous pyomyositis in a RA patient treated with anakinra.

Clinical and laboratory predictors of distinct histopathogical features of lupus nephritis.

The authors aimed to explore whether distinct clinical, serological, and urinalysis findings are associated with specific histological classes of lupus nephritis. Clinical and laboratory features were recorded at the time of clinical diagnosis from 297 consecutive patients with biopsy-confirmed lupus nephritis. Univariate and logistic regression analyses were performed and a risk score was developed to estimate the risk for developing different classes of lupus nephritis. Variables independently associated with class II included absence of malar rash, negative anti-dsDNA, and ≤5 urine leucocytes/high power field (hpf); with III/IV: age at nephritis diagnosis ≤32 years old, presence of musculoskeletal features, new-onset hypertension, positive anti-dsDNA, >5 urine leucocytes/hpf, creatinine >1.2 mg/dL, cellular casts >1/hpf, and absence of nephrotic range proteinuria; with V: age at nephritis diagnosis >32 years, malar rash, absence of musculoskeletal complaints or serum C3 hypocomplementemia, nephrotic range proteinuria, and ≤9 urine erythrocytes/hpf. A risk predictive score of specific histological classes was calculated for each patient. Associations between 2, 3 or more risk factors with specific histological classes were also revealed [Odds ratios (95% confidence interval) (≥2 risk factors) was 6.7 (2.8-17.4) for class II nephritis, 15.6 (5.1-47.8), and 8.2 (3.6-19.0) for classes III/IV and for class V, respectively (≥3 risk factors)]. The identification of independent factors associated with specific classes of lupus nephritis can provide guidance in selecting specific therapeutic modalities, particularly in cases in which renal biopsy is contraindicated.

Cancer diagnosis in a cohort of patients with Sjogren's syndrome and rheumatoid arthritis: a single-center experience and review of the literature.

BACKGROUND: With the development of modern therapies and better care of patients with autoimmune rheumatic diseases (ARDs) increased survival has been achieved. However, ARDs may share an association with risk of lymphomas and solid tumors. The increased cancer risk in these patients is mainly due to high inflammatory activity and severity of disease, rather than the immunosuppressive therapy. PATIENTS AND METHODS: We studied the coexistence or later development of cancer with ARDs in a retrospective audit of a reference university hospital and critically reviewed published literature. Fourteen out of 1,730 patients with rheumatoid arthritis (RA) and Sjogren's syndrome (SS) followed-up at the University Hospital of Ioannina over the last 33 years developed secondary malignancies, both solid tumors and lymphomas. RESULTS AND CONCLUSION: The most frequent cancer associated with ARDs is diffuse large B-cell lymphoma (DLBCL). The average risk of lymphoma in RA may be composed of a markedly increased risk in patients with most severe disease. Solid tumors were presented mainly in RA patients and renal cell carcinoma was the most frequently found.