Lynn, Michaela

Singh, Neena

Woolley, Andrew

English

AJOL - African Journals Online

South Sudan Medical Journal                                                                                               Vol 7. No 3. August 2014 62mAIN ARtICLESIntroductionSarcoidosis is a multisystem granulomatous disease of  unknown cause. It occurs worldwide but there are higher incidences in certain racial groups, being three to four times more common in African-Americans [1]. It can also aggregate in families. Most patients do not need treatment and the disease often regresses spontaneously, but a minority have potentially life-threatening progressive organ dysfunction; these patients need active management including oral corticosteroids. The poorest prognosis, in terms of  chronicity and fatality, is for African Americans [2]. This case illustrates that acute sarcoidosis can be a difficult diagnosis to make.Clinical assessmentA 59 year old man of  white Australian origin, who had been living in the UK for the last 35 years, presented to the medical admission unit of  our hospital with a cough, rash and pyrexia. The cough had been present for 3 months, increasing over the preceding 2 weeks, and was productive of  clear sputum. He gave a 10 day history of  an erythematous rash which involved all four limbs and the upper trunk, sparing the palms and soles. The patient also complained of  widespread arthralgia, with variable joint swelling, tenderness and stiffness. There was no history of  night sweats, weight loss or haemoptysis. His past medical history included fusion of  the left ankle joint following an injury at the age of  fifteen; he was otherwise fit and well. He was not on any regular medications and had no known drug allergies. He drank alcohol occasionally and had never smoked. He worked as an engineer. His last overseas travel was to the Australian outback in Queensland four years previously. He had no pets or significant animal exposure and no recent insect bites.On examination, his temperature was 37.3 0C and other observations were normal. There were no added heart sounds and vesicular breath sounds were heard throughout. There was bilateral pedal pitting oedema to below knee level. There were no peripheral stigmata of  vasculitis or palpable lymphadenopathy present. The abdomen was soft and non-tender with no organomegaly or palpable masses. The neurological examination was unremarkable. There was bilateral conjunctival injection. Dermatological Case study: sarcoidosisAndrew Woolleya, Michaela Lynnb and Neena Singhba Consultant Respiratory Physician, St Mary’s Hospital, Isle of Wight , UKb Foundation Year 1 doctors; St Mary’s Hospital, Isle of Wight , UKCorrespondence: Michaela Lynn kaelalynn83@hotmail.comexamination revealed multiple 1-2 mm erythematous macules on the arms, legs and upper trunk. There was a marked erythematous area on the right ankle and a firm tender swelling with surrounding erythema just distal to the left elbow. The latter had developed spontaneously on the day of  admission. During the following 30 days of  the patient being in Description Value RangeHaemoglobin 155g/l 135-185MCV 86 fl 78-100Platelets 289 x109/l 140-400Haemoglobin 155g/l 135-185MCV 86 fl 78-100Platelets 289 x109/l 140-400White cell count 10.9x 109/l 4.0-11.0Neutrophils 8.6 x109/l 2.0-7.5Lymphocytes 1.1 x109/l 1.0-3.5Urea 3.7 mmol/l 2.5-7.8Sodium 132 mmol/l 133-146Potassium 4.3 mmol/l 3.5-5.3Chloride 93 mmol/l 95-108Creatinine 82 μmol/l 60-110Estimated GFR 89 mL/min/1.73m2 >90CRP 174.9 mg/l 0.0-5.0Calcium 2.26 mmol/l 2.20-2.60Phosphate 1.14 mmol/l 0.80-1.50Adjusted Calcium 2.39 mmol/l 2.20-2.60Tot. Bilirubin 12 μmol/l <21Alkaline Phosphatase 95 U/L 30-130Total Protein 78 g/l 60-80Albumin 37 g/l 35-50Globulin 41 g/l 20-40Alanine Aminotransferase 45 U/L <40Table 1. Blood results on admission Vol 7. No 3. August 2014                                 South Sudan Medical  Journal 63mAIN ARtICLEShospital, he spiked temperatures daily, ranging from 37.3◦C to 38.6◦C. He also complained of  widespread arthralgia, reporting variable joint swelling. Towards the end of  his admission, the patient developed multiple, red, round, warm nodules on the postero-medial aspects of  the lower limbs, reminiscent of  erythema nodosum. InvestigationsInitial Investigations Routine bloods tests revealed a mildly raised neutrophil count and an elevated CRP. Renal function was not impaired and all electrolytes were within the normal range. Bone profile was stable – see Table 1.ECG showed left axis deviation. Urinalysis was negative for nitrites, leucocytes and blood. Repeated blood and sputum cultures showed no growth. Auramine stain was negative. Specific InvestigationsIn view of  the history of  travel in the Australian outback, relevant infectious diseases were investigated: Malarial screen, ASO titre, HIV and serology for Brucella, Chikungunya virus, Cytomegalovirus (CMV), Dengue virus, Epstein–Barr virus (EBV), Hepatitis B surface antigen, Hepatitis C antibody, Lyme, Mycoplasma, Orientiatsutsugamushi, Q fever, Ross River Fever, Spotted fever and Syphilis but all were negative. Other immunological and biochemical investigations were unremarkable – see Tables 2 and 3. The chest radiograph did not show any evidence of  infection and was thought to be normal – see Figure 1.CT of  the chest/abdomen/pelvis showed widespread lymph node enlargement throughout the mediastinum, suggesting possible sarcoidosis – see Figure 2.HistologyEndobronchial ultrasound (EBUS) guided needle aspiration of  the sub-carinal lymph node showed large numbers of  lymphocytes and scattered histocytes with no malignant cells or well-formed granulomas. Biopsy of  the skin lesions was considered but they were beginning to resolve before this could be done. Differential diagnosisInitial working diagnosis was cellulitis of  the right leg. Septic arthritis of  the right ankle was excluded by the Orthopaedic team.Differential diagnoses could be categorised into four broad areas:Description Value RangeESR 70 mm/hrProtein electrophoresis Inflammatory responseSerum IgA 2.73 g/l 0.80-4.00Serum IgG 13.77 g/l 5.30-16.50Serum IgM 0.80 g/l 0.50-2.00INR 1.0 0.8-1.2Beta-2 microglobulin 3.3 mg/l 1.2-2.4LDH 499 IU/l 225-425CA 19-9 32 KU/L 0-35CEA 0.9 μg/l 0.0-2.5AFP 1.2 KU/L 0.0-10.0Table 3. Chemical PathologyDescription Value RangeANA Ratio 2.4 < 1.0ANCA NegativeRheumatoid factor 57 IU/ml 0-25Table 2. ImmunologyInfections including demographic specific infections; 1. bacterial infections including TB, Rheumatic fever, mycoplasma and typhoid; viral infections including HIV, EBV, CMV and hepatitisImmune-mediated causes including sarcoidosis, 2. vasculitis, SLE, reactive arthritis, psoriatic arthritis and amyloidosisMalignancy including lymphoma, leukemia and 3. metastatic cancerOther causes of  unexplained pyrexia such as 4. Figure 1. Anterior-posterior chest radiographSouth Sudan Medical Journal                                                                                               Vol 7. No 3. August 2014 64mAIN ARtICLESabscess, haematoma, drug fever, pulmonary emboli, hyperthyroidism and sub-acute thyroiditisFollowing the CT result, the patient was referred to the Respiratory team. A diagnosis of  probable sarcoidosis was made. This was investigated by an EBUS guided needle aspiration which was performed in a regional tertiary hospital. Treatment and ProgressInitial treatment was with flucloxacillin for cellulitis. However, the progression of  the rash from the lower legs to the thighs, the bilateral swelling of  the elbows and the development of  tender red nodules, raised a concern that he had had a reaction to the antibiotic. This in conjunction with the ongoing pyrexia and elevated CRP (see graphs 1 and 2) prompted a switch to second line antibiotics (doxycycline), although this was not associated with any change in these outcomes.Following a review by the Respiratory Team, antibiotic treatment was stopped and this did not cause any deterioration in the patient’s clinical state. Treatment with steroids was withheld until a histological diagnosis could be made. The EBUS lymph node aspiration cytology was supportive of  the diagnosis of  sarcoidosis. He was referred to an Ophthalmologist, as per American Thoracic Society guidelines [3], who diagnosed ocular hypertension but did not find anyevidence of  uveitis or visual impairment. The patient was booked for an urgent follow-up out-patient appointment to discuss findings, prognosis and treatment options. At follow-up 2 months after discharge, he was asymptomatic and on no treatment.CommentPyrexia of  unknown origin (PUO) was first defined in 1961 as a temperature greater than 38.3°C on several occasions, accompanied by more than three weeks of  illness and a failure to reach a diagnosis after one week of  inpatient investigation [4]. In adults, infections, cancer and auto-immune disorders account for most PUOs. However, drug fever, granulomatous diseases-including sarcoidosis, vasculitides, pulmonary emboli, hyperthyroidism and subacute thyroiditis are other important causes. Erythema nodosum (EN) is characterized by purple or red, tender nodules, which can occur anywhere but are usually on the anterior aspect of  the lower leg. Accompanying arthralgia occurs in more than half  of  patients. Streptococcal infection is the most common underlying cause. Granulomatous diseases, including sarcoidosis and tuberculosis must be considered. EN may correlate with a flare-up of  Crohn’s disease or ulcerative colitis. It can precede the diagnosis of  Hodgkin’s lymphoma and non-Hodgkin’s lymphoma. It can be drug induced. In many cases no cause is found. Bilateral hilar lymph node enlargement can be a feature of  infection (particularly fungal or mycobacterial organisms) or malignancy (e.g. lymphoma). However bilateral hilar lymphadenopathy is the most common radiological finding in sarcoidosis [5]; this should therefore remain high on the differential diagnosis list. Figure 2. CT chest/abdomen/pelvis with contrastGraph 2. Fluctuating CRP during admissionGraph 1. Fluctuating temperatures during admission Vol 7. No 3. August 2014                                 South Sudan Medical  Journal 65mAIN ARtICLESWith hindsight, the pattern of  clinical features including pyrexia, erythema nodosum and bilateral hilar lymphadenopathy were highly suggestive of  a clinical diagnosis of  sarcoidosis. A good quality postero-anterior radiograph on admission to hospital may have brought the diagnosis to light much earlier. ReferencesThomas K.W. and Hunninghake G.W. 2003 Sarcoidosis.1. JAMA; 289(24):3300-3303Annuzzi M.C., Rybicki B.A. and Teirstein A.S. 2007 2. Sarcoidosis New England Journal of  Medicine;357(21):2153-65American Thoracic Society. Statement on Sarcoidosis. 3. 1999. American Journal of  Respiratory and Critical Care Medicine;160:736-55 Petersdorf  R.G. and Beeson P.B. 1961 Fever of  unexplained 4. origin: report on 100 cases. Medicine (Baltimore) Feb; 40:1-30.Hyun J. P., Jung I.J.,Myung H. C.,Sun W. S., Hyo L. M., Jun 5. H. B., Dae H. H., Ki J. K., and Kyo-Young L. 2009 Typical and A typical Manifestations of  Intrathoracic Sarcoidosis. Korean Journal of   Radiology  Nov-Dec; 10(6): 623–631. ‘pRImARy motHER CARE ANd popULAtIoN’Authors: Glen Mola, Jim Thornton, Michael Breen, Colin Bullough, Hugh Philpott, Douwe Verkuyl, Priscilla Busynge, and Maurice King “This book should be in every health unit in Africa”, so said a highly critical and long experienced doctor and aid worker of  an earlier edition. Its purpose is to stop mothers dying and to reduce Africa’s maternal mortality.  Although primarily intended for ‘the better educated midwives’, it should be useful for the less educated ones, and indeed for the more enquiring  members of  the general public. It is also highly suitable for medical students, and indeed for trainee consultant obstetricians, since their cadres are presently becoming deskilled in Africa. We constantly have in our minds the isolated midwife who is trying to do her first destructive operation ‘out of  the book’ on a mother with obstructed labour in the middle of  the night, never having done one before. We describe all the family planning methods in detail, including particularly the postcoital ones. The novel feature of  the book is however its second chapter “How many children?” which deals with the problem of  communities exceeding the carrying capacity of  their local ecosystems. This is ‘demographic entrapment’, which requires an immediate reduction of  fertility, in ‘a crash demographic transition’. Since demographic entrapment is tightly taboo to demography, development economics and the UN agencies and NGOs, this chapter breaks entirely new ground. We hope it will be a growing point for future ‘community disentrapment programs’.The book is large with 28 chapters, two million words, and several pictures on almost every page. It is problem oriented, with detailed instructions as to how to deal with every difficulty that we have ever been able to find. The introduction contains a glossary of  all technical terms in narrative form.It was put together in an unusual way, over many thousands of  hours, by a doctor who is  not an obstetrician, putting himself  in the position of  the reader and then finding out what he should do and know. To reduce its cost, it is published without royalty, for the love of  Africa. The book can be purchased from Kennedy Chadeka, Acrodile Publishing Ltd, Nairobi, Kenya. www.acrodile.co.keNote the above review was provided by the publisher and does not necessarily reflect the opinion of  SSMJ.

Case study: sarcoidosis

https://www.ajol.info/index.php/ssmj/article/download/132629/122227

Case study: sarcoidosis

Abstract

Similar works

Full text

Available Versions

AJOL - African Journals Online