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Case Presentation : 

Case Presentation Dr. Ayman El- Deeb

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Name :- sameer B. male Pt. 45 Years old from Kom HAMADA ????? he is a clerk presented to my clinic a month ago complaining of dyspnea on mild effort & occasional bouts of cough on effort of mild degree. Dyspnea started 6 months ago & it is increasing in severity. Pt. has swollen legs.

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The Pt. gave a history of swelling of the left leg 10 years ago . By that time it was diagnosed as DVT for which he received 6 months of anticoagulant therapy without regression of the leg swelling Doppler study was –ve for venous occlusion . Two years back he started to have swelling of the other leg Although he felt heaviness of both legs, his condition did not affect his working ability much. 6 months ago the Pt. started to feel heaviness in the chest – shortness of breath & occasional cough during muscular effort , he had no expectoration

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By that time he was examined by some chest physicians and he had an X ray of the chest that showed mild pl. effusion on the Rt. Side.

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In spite of the free Doppler study on both lower limbs. His condition was diagnosed as possible P.E. (pulmonary embolism). Again he was given anticoagulant therapy. In the meantime he was asked to have V/Q study of the lungs. Ventilation per fusion scan was normal & free of any defect.

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On examination of the patient :- Temp. 37Co pulse 90/ m Bp 140 / 80 mmHg. Resp. rate 20 / m at rest. The pt. has grade III dyspnea according to (NYHA) Classification – he was not cyanosed he has no clubbing of fingers – Both legs are swollen and non pitting.

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Dorsalis pedis arterial pulsation was felt with difficulty on the dorsum of both feet. Heart ex:- was unremarkable Chest examination:- Free, only very few wheezy rhonchi were occasionally heard . C X R :- Free both lung fields. Free costophrenic angles on both sides . Normal size & configuration of the cardiac shadow.

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CBC:- Showed marked esinophilia 12% of the WBcs count. ECG unremarkable Echocardiography:- apart from mild diastolic dysfunction – it was normal. Lung functions:- were of restrictive pattern his vital capacity was 52% of predictive value & FVC WAS 32 % of predicted P.E.F. 33% OF predicted.

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On review of the investigations done before coming to my clinic TC-99 m DTPA aerosol and TC-99 MAA Ventilation / perfusion V/Q lung scan (Done on 1/7/2008) . The study was –ve for pulmonary embolism C X R P.A (Done on 20/8/2008) Showed :- Obliteration of the RT . Costophrenic angle Free lung apices Normal sized heart.

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The pt. was sent to the research institute of medical entomology for serological examination of filariasis. The test was strongly positive.

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In the view of swollen legs – restrictive pulmonary functions – High eosinophil count – the strongly +ve serological test for filariasis the most likely diagnosis was T.P.E. Tropical pulmonary eosinophilia.

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The pt. was treated with (D.E.C.- diethylcarbmazine ) and Alzental. Rx D.E.C. 6 tab. State 6 tab. After 15 days 6 tab . After One month 6 tab. Every 6 month Alzental 2 tab. State 2 tab. After 15 days 2 tab . Every 6 months

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These drugs were provided and given to the pt. from the medical entomology institute in Dokki Cairo. These doses were recommended by prof. Nadia El Deeb (parasitology DPT. Cairo university) and prof. M. Ismaeel (Head of medical entomology institute) This in addition to corticosteroids. Prednisolon 20 mg daily for 2w. And then 10mg daily for the length of treatment course.

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After a month of that treatment the pt. showed a good clinical improvement. Eosinophil count dropped to 7% of the WBcs count Lung function test was repeated and it showed VC of 70 % of predicted and FVC of 63% of predicted values.

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T.P.E It is a rare syndrome but a well recognized condition – characterized by pulmonary infiltrates , dyspnea , occasional wheeze and marked peripheral esinophilia. Only a small percentage < 0.5% of pts. Infected with filariasis develop this reaction.

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The diagnostic criteria for T.P.E . Include. History supportive of exposure to lymphatic filariasis Pulmonary symptoms in the form of dyspnea –wheeze and restrictive lung functions. High peripheral eosinophilia E levated IGE High titer for antifilarial antibodies Peripheral blood –ve for microfilaria Clinical response to D.E.C.

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Radiological findings are non specific with normal CXR in up to 20%

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There is no universal treatment guide lines available till now, but D.E.C remains the main therapeutic agent. Early recognition and treatment with diethyl carbemazine is important as delay before treatment may lead to progressive interstitial fibrosis and irreversible impairment.

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2 nd Case Presentation By Ayman El- Deeb

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Name hamdeia El Akraa Female PT. 42 years old from Ekhnaway TANTA she presented to my clinic complaining of chronic cough and shortness of breath on mild exertion.

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The condition started gradually over the last 7 years with progressive dyspnea – dry cough – pruritus – hardening of the skin with ulcers on the tip of the fingers and arthralgia.

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On examination of the PT:- Pulse 100/ m Bp 130/80 mm Hg resp.rate 25/m The pt.’ s skin looked tight, indurated and shiny – there are areas of hyper – pigmentation and hypo pigmentation. Skin corrugation around the mouth with reduced mouth opening (microstomia)

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Skin of the hands appeared shiny & tight with sclerosis of the fingers & few painful ulcers on their tips.

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Areas of telangiectasia over the nose and root of the neck were seen.

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Heart examination: Normal heart beats all over the heart ,Accentuated 2nd sound over the pulmonary area. Chest examination: Coarse consenating crepitations were heard over both lung bases mainly on the back.

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Chest X ray: showed. Bilateral basal micro nodular shadows.

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C/T Chest showed bilateral diffuse interstitial and alveolar opacities. Markedly dilated distal esophagus.

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Lung functions:- are of restrictive pattern VC is 50% of predicted FVC is 50% of predicted PEF is 28% of predicted

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Lab. Investigations:- Mild anemia Strongly + ve ANA

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The condition was diagnosed as systemic sclerosis – It is a rare condition The prevalence of the disease is reported to be 13 -105 per million in north America and Europe The incidence of ssc is between 2.6 to 20 per million / per year It affects all races and is slightly higher in blacks than in whites Female / male ratio was reported as 3:1

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The disease took it’s name from the Greek words (Skleros means hard) & (Derma means skin) Hippocratis described the disease – Carlo Curzio in 1752 gave a detailed description of patients with hard skin in Naples.

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Giovambattista gave these patients with hard skin the name of scleroderma in 1945. Robert H. Gaetz described in detail the progressive nature of this systemic disease.

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Is a clinically heterogeneous generalized disorder which affects the connective tissue of the skin and internal organs such as G IT - lung and genitourinary tract- the disease is characterized by inflammation and progressive tissue fibrosis , occlusion of the microvasculature by excessive production and deposition of type I & III collagen fibers and matrix proteins. SSC

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In the skin it causes Hardening Telangiectasia Pigmentation GIT GERD Dysphagia due to hypo motility Peptic esophagitis SSC

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Lungs:- It causes lung fibrosis and pulmonary hypertension. CVS:- It causes raynaud’s disease palpitation – pulmonary hypertension – Telangiectasia – Rt - Sided failure Kidneys:- Protinuria – uremia – arterial hypertension . Genitalia :- Erectile dysfunction Endocrines:- Hypothyroidism Muscles and joints:- Polymyositis – Arthritis – juxta articular osteoporosis.

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In 2004 Maricq & valter proposed classification for scleroderma spectrum disease is as follows: Type I :- Diffuse skin involvement proximal to elbows / knees includes trunk. Type II:- Intermediate skin involvement proximal to the metacarpal / phalangeal metatarsal phalangeal joints – distal to the elbows and knees (Trunk not involved) Type III :- digital sclerodactyly only

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Type IV :- Scleroderma sine scleroderma (Capillary pattern – Visceral involvement no telangiectasia – no anticentromere antibodies. Type V:- undifferentiated connective tissue disease. Type VI :- Crest syndrome – No skin involvement or sclerodactyly only telangiectasia is required.

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Treatment Skin pruritus and thickening :- with moisturizers and anti histamines penicillamine. Raynaud phenomenon :- are treated with ca channle blockers – Bosentan- local nitrates – antiplatelet GIT:- H2 blockers – proton pump inhibitors and prokinetic drugs. Chest:- Bosentan (endothelin receptor antagonist for pulmonary hypertension). For I.P.F. Immuran glucorticoids n acc.

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Thank you