Psoriatic arthritis (PsA) is a chronic and often progressive inflammatory disease, occurring in up to 30% of patients with psoriasis. Assessment of patients with PsA requires consideration of all disease domains, including peripheral arthritis, axial disease, enthesitis, dactylitis, skin psoriasis, psoriatic nail disease, uveitis and inflammatory bowel disease. Co-morbidities and related conditions should all be considered including: obesity, metabolic syndrome, cardiovascular disease, anxiety/ depression, liver disease, chronic infections, malignancy, osteoporosis, fibromyalgia and reproductive health. K E Y W O R D Sassessment, diagnosis, psoriatic arthritis, treatment Psoriatic arthritis (PsA) is a chronic and often progressive inflammatory disease, occurring in up to 30% of patients with psoriasis. It is a heterogeneous condition with musculoskeletal involvement, including peripheral arthritis, enthesitis, dactylitis and axial involvement in addition to skin psoriasis, psoriatic nail disease and uveitis (Acosta
Introduction/Background Systemic lupus erythematosus (SLE) is a complex autoimmune condition which can present with a wide variety of clinical features. This variation can lead to delayed diagnosis or misdiagnosis. Gastroenetrological pathology in SLE is rare but primary autoimmune hepatitis, pancreatitis, lupus enteritis, cholestatic biliary disease and protein losing enteropathy have all been reported. We would like to share the first known presentation of SLE as cholangiocarcinoma. Description/Method A 62-year-old, previously fit and well, gentleman presented with a 1 month history of painless jaundice, a palpable gallbladder (Courvoisier syndrome), dark urine, pale stool, generalised rash, pruritus, weight loss and anorexia. The presence of these red flag features raised concerns. Initial investigation with Endoscopic retrograde cholangiopancreatography (ERCP) revealed distal common bile duct (CBD) stricture which was further explored with computerised tomography of thorax, abdomen, pelvis and magnetic retrograde cholangiopancreatogram (MRCP) both raising the suspicion of a cholangiocarcinoma in the presence of constitutional symptoms. Several biopsies and brushings taken during multiple ERCP’s failed to identify clear mitotic cells, IgG 4 staining remained negative. Further investigation with Positron emission tomography – computerised tomography (PET-CT) showed significant FDG uptake around the upper part of the stent with final working diagnosis of Cholangiocarcinoma and a Whipple’s procedure was planned. In the interim, the patient developed swollen painful hands, scleritis and a yellow blistering rash. He was referred urgently onto the Rheumatology team after receiving the positive immunology results: ANA (1:1280), Anti double stranded DNA and Rheumatoid Factor (RF) along with hypocomplementemia (C3&C4). Discussion/Results Subsequent work up under rheumatology was done considering the possibilities of diagnosis of Systemic lupus erythematosus (SLE), Lupus related autoimmune hepatitis and reactive arthritis as a result of underlying malignancy. Patient’s immunology and other clinical features such as skin rash were strongly supportive of SLE as a primary diagnosis. He was initially treated with low dose prednisolone with dramatic improvement in arthritis and gradual improvement in liver function test. Following MDT discussions with the hepato-biliary team it was felt that lupus was the cause of the biliary stricture which settled well with steroid treatment. A final ERCP was arranged to remove the stent and take final brushings. Methotrexate was started at this point. His SLE remains well controlled on methotrexate and his liver function tests, including bilirubin, remain within the normal range. Patient has now been completely asymptomatic and is discharged from the care of hepatobiliary team with long term follow up for his SLE with rheumatology. Key learning points/Conclusion This is extremely rare first presentation of SLE presenting with painless jaundice and biliary stricture which improved both biochemically and clinically with treatment of SLE. The key learning points included: Urgent review by rheumatology can facilitate timely diagnosis and preventable admissions/interventions such as major intraabdominal procedure in the form of whipple’s disease planned in our case. Cancer remains a major cause of mortality and morbidity in rheumatological conditions like SLE. Hence initial diagnosis can become extremely difficult if an overlap between these two happens. Treatment options can be limited initially and steroids remain the main stay of treatment till mitotic lesions have been ruled out and disease modifying agents can be used without any contraindications. But disease control for our conditions remains challenging in presence of cancers.
Background/Aims In rheumatological practice it in not unusual to see patients with haematological involvement. We would like to present a very rare diagnosis of Erdheim-Chester disease presenting as retroperitoneal fibrosis and large vessel vasculitis who failed to respond to conventional treatments with steroids and methotrexate. Methods A 71-year-old man presented to haematology with retroperitoneal mass. This was investigated with CT thorax, abdomen and pelvis and a biopsy was performed which was negative for haematological malignancies. However, CT scan picked up thickening of aorta and a PET scan was performed subsequently confirming widespread aortitis and peri-renal mass thought to be consistent with retroperitoneal fibrosis. The patient was started on reducing course of oral steroids with a working diagnosis of retroperitoneal fibrosis with large vessel vasculitis. Results Subsequent staining for IGG-4 was negative and biopsy was not typical with IgG4 cells. The patient was started on high doses of oral steroids by haematologist with Methotrexate started subsequently by rheumatologist due to persistent changes on PET scan, high inflammatory markers and worsening of patient with generalised malaise, weight loss and fatigue. The patient failed to respond to uptitrated doses of methotrexate and was discussed with a specialist unit for consideration of anti-IL6 tocilizumab to control his large vessel vasculitis. On discussion of imaging in MDT, unusual appearance with sparing of ureters, splaying of peri-nephric fat, lack of IgG4 cells pointed towards an alternative diagnosis. Conclusion The patient was subsequently discussed in the national adult histiocytosis meeting with national and international haematologists. The clinical presentation, radiological appearance was thought to be classic of Erdheim-Chester disease, a rare form of adult onset histiocytosis. After further discussions with specialist centre the patient has been commenced on trametinib, a MEK inhibitor treatment (cell tissue negative for BRAF mutation). The patient has recently developed heart failure and has undergone right heart catheter which has found extremely rare pulmonary artery vasculitis too. Disclosure S. Raghuvanshi: None. B. Tarar: None. A. Shaaban: None.
Introduction: At present, the treatment of choice for appendicular masses is unclear. Recent studies claimed that conservative management of appendicular masses was safe in terms of frequency of perforation. However, there is controversy in the existing literature.Objective: This research is designed to compare the results of early appendectomy versus conservative management of appendicular masses.Material and methods: It was a randomized controlled trial performed in the Combined Military Hospital, Lahore. The study lasted six months, from 01/03/2019 to 30/09/2019. It involved 60 patients of both genders aged between 16 and 70 years diagnosed with appendicular masses with an Alvarado score of 4-7. These patients were randomly divided into two treatment groups. In Group A patients, an early appendectomy was performed, while patients in Group B were managed conservatively. Outcome variables were the mean length of hospital stay and frequency of appendicular perforation.Results: The mean age of the patients was 26.8±11.9 years. There were 33 (55.0%) male and 27 (45.0%) female patients, with a male-to-female ratio of 1.2:1. The mean length of hospital stay was significantly longer in patients managed conservatively as compared to those undergoing early appendectomy (2.80±1.54 vs. 1.83±0.83; p=0.004). However, the frequency of perforation was not significantly higher in the conservative group as compared to the early appendectomy group (16.7% vs. 10.0%; p=0.448).Conclusion: Conservative management of patients with appendicular mass was associated with prolonged hospital stays, yet it was found equally safe in terms of frequency of appendicular perforation, which advocates conservative management of patients with appendicular mass, particularly in high-risk patients.
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