Anthony O'Regan

Hiatal hernias are correlated with increased severity of non-cystic fibrosis bronchiectasis.

Hiatal hernias (HH) are associated with gastro‐oesophageal reflux and may contribute to lung disease severity. We aimed to evaluate the prevalence of HH among stable non‐cystic fibrosis bronchiectasis (NCFB) patients and determine associations with disease severity.

Sarcoidosis complicating treatment with adalimumab for Crohn's disease

Dear Sir, Adalimumab is a humanised monoclonal antibody against tumour necrosis factor-alpha (TNF-α), with proven efficacy in several autoimmune diseases. We report the development of sarcoidosis with cutaneous and pulmonary involvement in a 37-year old white Irish male with refractory Crohns disease of 13-years, commenced on maintenance therapy with adalimumab for 2 years following surgery and unsuccessful remission with methotrexate (Fig. 1). To the best of our knowledge, this is the fourth reported case in the literature of sarcoidosis complicating TNF-α therapy in Crohns disease; the second with adalimumab, others reporting paradoxical sarcoidosis with infliximab and natalizumab.1–3 Figure 1 (a) Chest X-ray on presentation in 2011 showing new prominent hilar and mediastinal adenopathy with multiple …

Flushing out the diagnosis

### Learning Point for Clinicians Always consider the non-infectious differential of the SIRS. Measuring serum tryptase may be useful in the setting of shock, particularly when the presentation may be due to an anaphylactoid reaction. A 30-year-old man presented with a 4-h history of severe headache, vomiting, diarrhoea and epigastric pain. 1 week previously, he had undergone a left hip arthroscopy in another hospital. He had no other medical history and his only medication was naproxen for post-operative pain. His blood pressure was 70 mmHg systolic, pulse rate 130 bpm, respiratory rate 24 bpm and temperature was 38.3°C. Physical examination was notable for a diffuse erythematous, blanching rash involving his face and trunk, conjunctival suffusion and mild abdominal tenderness. Laboratory studies were normal apart from a leucocytosis (18.1 × 109/l) and a creatinine of 149 µmol/l. The provisional diagnosis was of toxic shock syndrome (TSS) secondary to peri-operative staphylococcal infection …

Taught a lesson by taut skin

A 45 year old man with a history of ulcerative colitis presented to his general practitioner because of Raynaud’s phenomenon and itchy tight skin that had affected his hands and elbows bilaterally over the preceding few months. He was referred to a rheumatologist, who performed a series of immunological tests. The results included a positive antinuclear antibody staining pattern on immunoflourescence, with anti-RNA polymerase III antibodies identified on further testing. Tests for anti-centromere and anti-topoisomerase I (anti-Scl 70) antibodies were negative. The rheumatologist diagnosed a systemic connective tissue disorder. When the skin symptoms worsened over the next few weeks the patient started to use over the counter ibuprofen to ease the pain associated with his tight skin. He subsequently presented to the emergency department with fatigue. His blood pressure was found to be 210/120 mm Hg. In addition to the skin abnormalities, bibasal fine inspiratory crepitations were detected on physical examination. Routine laboratory testing showed haemoglobin 75 g/L (reference range 130-180), creatinine 475 µmol/L (80 µmol/L one month previously (80-110), and lactate dehydrogenase 357 U/L (70-250). Electrocardiography showed T wave inversion in the lateral leads. He was excreting 1.15 g of protein in his urine per 24 hours and haematuria was detected on dipstick urinalysis. ### What is the diagnosis? #### Short answer Scleroderma renal crisis, which results in a hypertensive emergency.1 #### Long answer Our patient had diffuse cutaneous scleroderma. Figures 1⇓ and 2⇓ show taut shiny skin on his forearm and hands, with areas of hypopigmentation on the extensor surfaces. The skin was so tight that he developed contractures at the elbows (fig 1 shows the position of maximal …