A critical analysis of 57 cases of Hughes-Stovin syndrome (HSS). A report by the HSS International Study Group (HSSISG).

BACKGROUND: Hughes-Stovin syndrome (HSS) is a systemic disease characterized by widespread vascular thrombosis and pulmonary vasculitis with serious morbidity and mortality. The HSS International Study Group is a multidisciplinary taskforce aiming to study HSS, in order to generate consensus recommendations regarding diagnosis and treatment. METHODS: We included 57 published cases of HSS (43 males) and collected data regarding: clinical presentation, associated complications, hemoptysis severity, laboratory and computed tomography pulmonary angiography (CTPA) findings, treatment modalities and cause of death. RESULTS: At initial presentation, DVT was observed in 29(33.3 %), thrombophlebitis in 3(5.3%), hemoptysis in 24(42.1%), and diplopia and seizures in 1 patient each. During the course of disease, DVT occurred in 48(84.2%) patients, and superficial thrombophlebitis was observed in 29(50.9%). Hemoptysis occurred in 53(93.0%) patients and was fatal in 12(21.1%). Pulmonary artery (PA) aneurysms (PAAs) were bilateral in 53(93%) patients. PAA were located within the main PA in 11(19.3%), lobar in 50(87.7%), interlobar in 13(22.8%) and segmental in 42(73.7%). Fatal outcomes were more common in patients with inferior vena cava thrombosis (p = 0.039) and ruptured PAAs (p < 0.001). Death was less common in patients treated with corticosteroids (p < 0.001), cyclophosphamide (p < 0.008), azathioprine (p < 0.008), combined immune modulators (p < 0.001). No patients had uveitis; 6(10.5%) had genital ulcers and 11(19.3%) had oral ulcers. CONCLUSIONS: HSS may lead to serious morbidity and mortality if left untreated. PAAs, adherent in-situ thrombosis and aneurysmal wall enhancement are characteristic CTPA signs of HSS pulmonary vasculitis. Combined immune modulators contribute to favorable outcomes.

Autoantibodies to extractable nuclear antigens (ENAs) pattern in rheumatoid arthritis patients: Relevance and clinical implications.

OBJECTIVES: To study the frequency of different autoantibodies to extractable nuclear antigens (ENAs) in rheumatoid arthritis (RA) patients and to correlate findings with clinical manifestations, disease activity and radiological damage. METHODS: A total of 230 RA patients were included and 75 healthy controls. In all patients rheumatological assessment was done and routine laboratory investigations and immune profile were performed in both patients and controls, including: RF, ACPA, ANA and anti-ENAs (Ro/SSA, La/SSB, U1-RNP, anti-Jo-1 and anti-Sm). Radiological damage was scored using Sharp/van der Heijde, and disease activity was evaluated by DAS28-ESR and DAS28-CRP. RESULTS: RF was positive in 101 (43.9%), ACPA in 220 (95.7%), ANA in 58 (25.2%), anti Ro in 31 (13.5%), anti-La in 10 (4.3%), anti-Jo1 in 5 (2.2%) and anti-RNP in 2 (0.9%). Anti-Ro/SSA positively correlated with sicca symptoms (p=.02), RF titer (p<.001), ANA (p<.001), DAS28-ESR (p=.026), and DAS28-CRP (p=.003). Anti-La antibodies correlated positively with SJC (p=.001), TJC (p=.001), ANA (p<.001), DAS-28 ESR (p=.007). Anti-Jo-1 correlated positively with interstitial lung disease (ILD) (p≤.001), RF titer (p=.037) and ANA (p≤.001). Anti-RNP antibodies correlated positively with disease duration (p≤.001), ACPA titer (p≤.001) and ANA (p=.014). In the controls ANA was positive in two (2.7%), anti-Ro in three (4%), and none of the controls tested positive for other autoantibodies. CONCLUSIONS: In RA patients, positive ANA is frequent and positively associated with anti-Ro, anti-La and anti-Jo1 autoantibodies. Screening for autoantibodies against other anti-ENAs seems mandatory in RA patients especially when ANA is positive. RA cases with positive Anti-Jo-1 may develop anti synthetase syndrome and ILD.

Gadolinium-enhanced MRI features of acute gouty arthritis on top of chronic gouty involvement in different joints.

The aims of the current study are to describe gadolinium-enhanced MRI features of an acute flare of established gouty arthritis in different joints and to examine a possible association between serum uric acid and MRI signs indicative of ongoing inflammation and/or structural joint damage as well as association with disease characteristics and laboratory findings. Twenty-seven male patients with established chronic gout agreed to participate, mean age 47.6 years, and mean disease duration in months 43.2 (±31.8). For all patients, detailed demographic, disease characteristics, and laboratory findings were obtained and correlated with MRI findings. In 27 patients with established gout, a total of 50 MRI studies were performed of the following joints: feet joints (n = 23), ankles (n = 18), knees (n = 5), and hand and wrist joints (n = 4). MRI revealed capsular thickening in 19 patients, bone marrow edema (BME) in 15, soft tissue edema (STE) in 20, joint effusion in 21, bone erosions in 17, cartilaginous erosions in 4, and tenosynovitis in 9 cases. In 17 cases, tophaceous lesions were found. Post contrast MRI showed synovial thickening in seven cases. Positive correlations were observed between serum uric acid levels and the following MRI findings: capsular thickening (r = 0.552, p = 0.003), BME (r = 0.668, p ≤ 0.0001), STE (r = 0.559, p = 0.002), and tenosynovitis (r = 0.513, p = 0.006). Using MRI in chronic gout, important features can be detected like BME, minute cartilaginous erosions, and hypertrophic synovial inflammation in post contrast MR images. Serum uric acid (SUA) was positively correlated with capsular thickening, BME, STE, and tenosynovitis.