Clinical immunology

    Clin" rel="nofollow">inical immunology         In the followin" rel="nofollow">ing pages two different cases of autoimmune disorders are outlin" rel="nofollow">ined- The case studies in" rel="nofollow">include a range of presentin" rel="nofollow">ing symptoms with any relevant previous history, or family history if appropriate- Also in" rel="nofollow">included is a range of results from recently performed laboratory in" rel="nofollow">investigations, carried out on either the patient or biological samples from the patient- a) You are required to arrive at a possible diagnosis for each of the patients, by assessin" rel="nofollow">ing and evaluatin" rel="nofollow">ing the significance of the pathology results, in" rel="nofollow">in light of the history of the patient- All presentin" rel="nofollow">ing signs and symptoms need to be fully explain" rel="nofollow">ined- b) Word count = 1,500 +/- 10% for each case- c) You need to explain" rel="nofollow">in the significance of the results of all of the laboratory in" rel="nofollow">investigations, in" rel="nofollow">in order to justify your conclusions and diagnoses- d) You should outlin" rel="nofollow">ine any further in" rel="nofollow">investigations (if any) which should, in" rel="nofollow">in your opin" rel="nofollow">inion, be performed to confirm the diagnoses- e) You should discuss the potential therapies for the treatment of the conditions you conclude to be present in" rel="nofollow">in each case and outlin" rel="nofollow">ine the mechanisms of the therapies- Case Study # 1 Mr Brown, aged 58 years, a retired min" rel="nofollow">iner, presented himself at his local hospital’s Accident & Emergency Department with difficulty of gettin" rel="nofollow">ing his breath- He also complain" rel="nofollow">ins of an in" rel="nofollow">increasin" rel="nofollow">ing weakness in" rel="nofollow">in his shoulders- He complain" rel="nofollow">ins in" rel="nofollow">in fin" rel="nofollow">indin" rel="nofollow">ing it difficult to raise his arms above shoulder level- He has found it in" rel="nofollow">increasin" rel="nofollow">ingly difficult over the last few months in" rel="nofollow">in pullin" rel="nofollow">ing himself up to a standin" rel="nofollow">ing position from his amichair- He also has difficulty in" rel="nofollow">in carryin" rel="nofollow">ing out fairly light manual work, such as sweepin" rel="nofollow">ing out his pigeon ducket (followin" rel="nofollow">ing his retirement he has recently taken up breedin" rel="nofollow">ing and racin" rel="nofollow">ing pigeons-) The newly qualified SHO in" rel="nofollow">in casualty notices that Mr Brown also has a ptosis, which Mr Brown says has developed ‘fairly recently’- The SHO decides to admin" rel="nofollow">inister a sin" rel="nofollow">ingle dose of a drug named edrophonium - he sees that there is an almost immediate improvement in" rel="nofollow">in Mr Brown’s ptosis- A range of blood samples are taken and sent off for analysis: Full Blood count: all parameters within" rel="nofollow">in normal range Erythrocyte Sedimentation Rate: 4mm/hr IgG precipitin" rel="nofollow">ins again" rel="nofollow">inst avian protein" rel="nofollow">ins: Negative (countercurrent electrophoresis) HLA haplotype: HLAB7, DR2- Routin" rel="nofollow">ine Autoantibody Screen: Negative except an anti-nuclear antibody present at a dilution of 1110. Anti AchR: result to follow later- Additional question: What is the connection between this disorder and the deadly snake, the Thai Cobra?     Case Study # 2 Mr Black, aged 64, a retired process worker presented at his GP’s surgery with a fairly complex array of complain" rel="nofollow">ints and symptoms- He complain" rel="nofollow">ined of general aches and pain" rel="nofollow">ins in" rel="nofollow">in his join" rel="nofollow">ints over the last few months, main" rel="nofollow">inly affectin" rel="nofollow">ing his wrists and ankles- He had put this down to “arthritis and advancin" rel="nofollow">ing years’- He then noticed that the tips of his fin" rel="nofollow">ingers were becomin" rel="nofollow">ing whitened and his fin" rel="nofollow">ingers had begun to feel cold- He states that the skin" rel="nofollow">in on his fin" rel="nofollow">ingers and hands seemed to becomin" rel="nofollow">ing tighter, and the wrin" rel="nofollow">inkles seemed to be disappearin" rel="nofollow">ing from his hands- In the last few weeks the fin" rel="nofollow">ingers on his left hand had started to become at first discoloured and had become in" rel="nofollow">increasin" rel="nofollow">ingly blackened- On further questionin" rel="nofollow">ing by the doctor, Mr Black complain" rel="nofollow">ined of dryness and soreness of the mouth and eyes, and reported an in" rel="nofollow">increasin" rel="nofollow">ing difficulty in" rel="nofollow">in swallowin" rel="nofollow">ing (dysphagia)- He was also embarrassed to admit that he had become in" rel="nofollow">increasin" rel="nofollow">ingly flatulent and had suffered fairly severe diarrhoea in" rel="nofollow">in the last few weeks, followed by spells of constipation, before his presentation at the surgery- A number of blood samples were taken for laboratory in" rel="nofollow">investigation and Mr Black was referred to the local hospital for X-ray in" rel="nofollow">investigation- At the hospital a skin" rel="nofollow">in biopsy sample was also taken for processin" rel="nofollow">ing and in" rel="nofollow">investigation: Full blood count: AII parameters were within" rel="nofollow">in the normal range Erythrocyte Sedimentation Rate: 35 mm/hr Serum electrophoresis: Indicated a marked, polyclonal, hypergammaglobulin" rel="nofollow">inaemia- Serum Immunoglobulin" rel="nofollow">in levels: IgG = 35 gII, IgA = 8 gII, IgM = 12 gII X-Rays: ‘Arthritic’ join" rel="nofollow">ints showed a thickenin" rel="nofollow">ing of the periarticular soft tissues and juxta-articular osteoporosis- Urin" rel="nofollow">ine examin" rel="nofollow">ination: Protein" rel="nofollow">inuria- Skin" rel="nofollow">in biopsy analysis: Shows a thin" rel="nofollow">innin" rel="nofollow">ing of the epidermis with Ig deposition at the dermal-epiderrnal border. Arteriolar fibrosis was also evident- Autoantibody screen: a) Anti Nuclear Antibody = Positive at a titre of 11320. This antibody showed an anti-nucleolar pattern of stain" rel="nofollow">inin" rel="nofollow">ing on immunofluorescence- b) Anti Salivary Duct antibody: Positive-