We report the case of a patient with Rheumatoid Arthritis [RA] presenting with clinical-pathological and radiological features of Pulmonary Lymphomatoid Granulomatosis (PLG). This is a rare lung disorder characterized by multiple nodular lesions with lymphocytic invasion of vascular walls. We present one such case of PLG secondary to Methotrexate and Azathioprine therapy, who was successfully treated with Steroids and Rituximab. We wish to highlight the importance of lung biopsy in the diagnosis and the use of rituximab as a treatment modality for RA as well as PLG.Electronic supplementary materialThe online version of this article (doi:10.1186/2193-1801-3-751) contains supplementary material, which is available to authorized users.
We report the case of a man presenting with clinical features of a mononeuritis multiplex, a perinuclear-ANCA (p-ANCA), and a renal biopsy suggestive of Wegener granulomatosis (WG). We wish to highlight this case as a learning point for clinicians as WG rarely presents in this form, and can be easily overlooked as a cause of mononeuritis multiplex.
SUMMARYPeriodic thyrotoxic paralysis is a genetic condition, rare in the West and in Caucasians. Thyrotoxicosis, especially in western hospitals, is an easily overlooked cause of sudden-onset paralysis. We present a case of a 40-yearold man who awoke one morning unable to stand. He had bilateral lower limb flaccid weakness of 0/5 with reduced reflexes and equivocal plantars; upper limbs were 3/5 with reduced tone and reflexes. ECG sinus rhythm was at a rate of 88/min. PR interval was decreased and QT interval increased. Bloods showed potassium of 1.8 mEq/L (normal range 3.5-5), free T4 of 29.2 pmol/L (normal range 6.5-17) and thyroidstimulating hormone (TSH) of <0.01 mIU/L (normal range 0.35-4.94). Random urinary potassium was 8.8 mEq/L (normal range 12.5-62.5). The patient was admitted initially to intensive therapy unit and given intravenous potassium. His symptoms resolved within 24 h. He was diagnosed with thyrotoxic periodic paralysis. He was discharged on carbimazole and propanolol, and follow-up was arranged in the endocrinology clinic. BACKGROUND
We hereby present a case of anaemia in a 73 years old patient with known past medical history of diffuse systemic scleroderma, who presented with acute onset of dizziness and haemetemesis. Blood tests revealed sudden drop of haemoglobin and an urgent gastroscopy revealed gastric antral vascular ectasia (GAVE) or “watermelon stomach”. GAVE is a rare but well recognised cause of acute bleeding in systemic scleroderma patients and should be kept as a differential diagnosis in the work up of anaemia in these patients.
We report a case of a 56-year-old female with a confirmed diagnosis of Dermatomyositis on muscle biopsy, which was refractory to 5 different disease modifying drugs as well as intravenous immunoglobulins and symptoms improved dramatically with a single course of Rituximab. We hereby wish to highlight the importance of Rituximab in this highly resistant case and that anti CD 20 biologic drugs can be considered as standard treatment protocol in refractory Dermatomyositis.
Interstitial lung disease is an important cause of mortality and morbidity in patients with systemic sclerosis (SSc). There are currently no recommended guidelines for management of these patients. This is probably due to the rarity of this condition, as well as clinical trials with only a small number of cases. There are published case report and case series along with the two main trials, viz. Scleroderma Lung Study and the Fibrosing Alveolitis Study, but again, there is no consensus on treatment protocols. In this report, we present a case of aggressive interstitial lung disease in a patient with SSc, which improved dramatically on treatment with intra-venous cyclophosphamide and high dose prednisolone therapy.
We describe the cases of three patients, under the care of the rheumatology service, who presented with osteonecrosis of the jaw whist on oral bisphosphonate therapy. The first case is of a 74-year-old woman with a 12 year history of sero-negative inflammatory arthritis, having been on oral steroids for 11 years, Methotrexate for the preceding 6 years, and oral bisphosphonates for 9 years. Clinical and radiographic examination revealed extensive jaw necrosis. The second patient was a 72-year-old woman with temporal arteritis, on long term oral steroids, and oral bisphosphonates presenting with jaw osteonecrosis. The third case is of an 81-year-old lady with a diagnosis of Polymyalgia Rheumatica on reducing dose of prednisolone along with calcium and vitamin D3 and oral bisphosphonate therapy as part of steroid induced prophylaxis guidelines. On reviewing the literature regarding bisphosphonate-associated osteonecrosis of the jaw, there is indeed recognition of this occurring with oral bisphosphonates. However, this is far less common than with intravenous preparations. Reports to the UK MHRA regarding adverse reactions have shown 53 cases of osteonecrosis of the jaw associated with oral bisphosphonates, but this is thought to represent under-reporting. We suggest consideration of patient counselling and consent, and preventive dental work prior to initiation of oral bisphosphonate therapy.
We assessed investigations and management protocols for patients starting long-term steroid therapy. Rheumatology, respiratory, hematology, oncology, and gastroenterology consultants were asked five questions designed to assess investigation and management protocols followed in dealing with 50-year-old male, 25- and 75-year-old female patients. The response rates were as follows: rheumatologists 11/14 (78.6%), gastroenterologists 19/31 (61.3%), respiratory physicians 13/26 (50%), hematologists 10/25 (40%), oncologists 6/21 (28.6%). In 50-year-old men, calcium + vitamin D was recommended by 6/11 (54.54%) rheumatologists, 7/13 (53.84%) respiratory physicians, 5/10 (50%) hematologists, 2/6 (33.33%) oncologists, and 13/19 (68.42%) gastroenterologists. Bisphosphonates were recommended by 3/11 (27.22%) rheumatologists, 8/13 (61.53%) respiratory physicians, 2/10 (20%) hematologists, and 5/19 (26.31%) gastroenterologists. Bone densitometry was considered by 4/11 (36.36%) rheumatologist, 3/13 (23.07%) respiratory physicians, 2/10 (20%) hematologists, 8/19 (42.10%) gastroenterologists. Calcium, phosphate, and alkaline phosphate were checked by 3/11 (27.27%) rheumatologists, 3/13 (23.07%) respiratory physicians, 2/10 (20%) hematologists, 3/6 (50%) oncologists, 6/19 (31.57%) gastroenterologists. In 25-year-old women, 4/11 (36.37%) rheumatologists, 3/13 (23.07%) respiratory physicians, 2/10 (20%) hematologists, 1/6 (16.66%) oncologists, and 4/19 (21.05%) gastroenterologists considered bone densitometry. Calcium, phosphate, and alkaline phosphatase were checked by 3/11 (27.27%) rheumatologists, 1/13 (07.69%) respiratory physicians, 3/10 (30%) hematologists, 3/6 (50%) oncologists, and 6/19 (31.57%) gastroenterologists. Calcium + vitamin D treatment was favored by 8/11 (72.72%) rheumatologists, 8/13 (61.51%) respiratory physicians, 5/10 (50%) hematologists, 2/6 (33.33%) oncologists, 14/19 (73.68%) gastroenterologists. Bisphosphonates were considered by 2/18 (18.18%) rheumatologists, 6/13 (46.15%) respiratory physicians, 1/10 (10%) hematologists, 1/6 (16.66%) oncologists, and 3/19 (15.78%) gastroenterologists. In 70-year-old women, calcium + vitamin D was recommended by 8/11 (72.72%) rheumatologists, 9/13 (69.23%) respiratory physicians, 5/10 (50%) hematologists, 2/6 (33.33%) oncologists, and 15/19 (78.94%) gastroenterologists. Bisphosphonates were considered by 9/11 (81.81%) rheumatologists, 13/13 (100%) respiratory physicians, 9/10 (90%) hematologists, 2/6 (33.33%) oncologists, and 18/19 (94.73%) gastroenterologists. Reloxifene was considered by 4/11 (36.36%) rheumatologists and 2/6(33.33%) oncologists. This survey demonstrates the lack of consensus in investigating and treating male and young female patients at risk, exceptions being elderly women.
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