wegener’s granulomatosis

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Wegener’s granulomatosis Dr. Sushanta Mahapatra Registrar MU-II , Mymensingh Medeical College Hospital, Bangladesh

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50 years male with

Wegeners granulomatosisDr. Sushanta MahapatraRegistrar MU-II , Mymensingh Medeical College Hospital, Bangladesh

PARTICULARS OF PATIENT:

Name: Md. Abdul MotalebAge: 45 yearsSex: Male Marital status: marriedOccupation: farmerReligion: IslamAddress: Nokla, SherpurDate of admission: 19/10/15Date of examination: 19/10/15

Chief complaints

Painful Red eyes for 3 months.Nasal blockage with occasional discharge for 2 monthsCough with blood mixed sputum for same duration.Fever for 20 days.

History of present illness: The patient states that he was reasonably well about 3 months back. Since then, he developed painful red eye and swelling of right eye with thicky discharge. Discharge was through out the day and more at morning after awaking from sleep, which was purulent and non foul smelling.

For this problem he attended several physicians and took some medication, but the condition became worse and gradually involved both eyes for last 2 months. For last 2 days his vision became severely impaired that he could not see anything.

Patient also complaints of Nasal blockage with occasionally watery nasal discharge for 2 months. For this complaints he consulted with physician and according to his advise he admitted in ENT dept. of MMCH.

Patient also stated that he has been suffering from cough for 2 months. It was initially dry and then became productive which was scanty in amount ,serous in nature and not foul smelled . For last 15 days he also notice that his sputum is occaisonally blood stained.

There is no history of chest pain, dyspnoea, vomiting, haematemesis or bleeding from any other site.

He also complaints of High grade , intermittent Fever for last 15 days which appeared almost every day and remain for about 1 hour, and subsided spontaneously. Highest recorded temperature was 103 F and fever is not associated with chills-rigor.

Patient also complaints of throat pain anf voice change for last 15 days, and difficulty in chewing for last 9 days, on query patient also mention that there is deviation of angle of mouth towards right side.

There is no history of nasal regurgitation, dysphagia or emotional labiality. His bowel and bladder is normal.

He also complaints of anorexia and significant weight loss during the course of the illness. There is no history of oral ulcer, skin rash, joint pain or swelling. Patient is normotensive and non diabetic.

History of Past illnessThere is no history of tuberculosis , jaundice or other significant illness.

Drug History During the course of this illness he attended several physician and took different medication, e.g analgesics, antibiotics, anti histamine , steroids, antibiotic eye drop and nasal decongestant.

PERSONAL HISTORY Patient is smoker and He smokes 25 sticks/day for last 25 years. He is Non alcoholic. There is no History of extra marital sexual exposure .

FAMILY HISTORY

He has 1 son, 3 daughter . None of his family member is suffering from this type of disease. They are healthy and enjoining sound health.

SOCIO-ECONOMIC HistoryHe comes from low socio-economic condition.Housing: Tin shade house.Sanitation: He use sanitary latrine.Water supply: He drinks from Arsenic free tube-well water.

Immunization history

He was immunized as per previous national immunization schedule.

GENERAL EXAMINATION Appearance ill lookingBody built averageDecubitus: on choice Co-operation : Well co-operative

Eye: Eyes are red, swollen and there is scleral injection , corneal Haziness present on both eyes.

Nose: There is depression of nasal bridge, and crusting on both nasal cavities.

Anaemia mild Jaundice : AbsentCyanosis : AbsentClubbing : AbsentKoilonychia : AbsentLeuconychia : AbsentOedema : AbsentDehydration Absent

skin condition is normal . Body hair distribution NormalBony tenderness absent Lymph node no lymphadenopathy .Thyroid gland not palpable

JVP- not raisedTemperature- 101 deg. Farenhite.Respiratory Rate- 16 breathsper minPulse 88/min, regular B.P 110/70 mm of Hg

Systemic examinationNervous system examination Higher psychic function test: Normal.

Cranial Nerves : There is bilateral 6 th nerve palsy Left sided Lower motor type of 7 th nerve palsy. Other cranial nerves are intact Motor function Test: No Abnormalities foundSensory Function Test: All sensory modalities intactCerebellar Function Test: No Abnormalities found

Respiratory System

INSPECTION

No abnormalities

PALPATIONTrachea: CentralCricosternal distance: NormalApex beat: Left 5th intercostal space 9 cm from midline, normal in character. Vocal fremitus: Normal.

PERCUSSION:Percussion Note: Resonance

Upper border of liver dullness in Right 5th intercostal space.

AUSCULTATION: Breath sound is vesicular

Added sound: There is coarse crepition over the upper and middle zone of both lungs, which is altered by coughing.

Vocal resonance: Normal.

Examination of Other system Reveals no abnormalities.

Salient feature:-Md. Abdul Motaleb, 45 years of age, farmer, normotensive, non diabetic, non alcoholic, smoker hailing from nokla, Sherpur admitted to MU-2, MMCH with the complaints of Painful Red eyes for 3 months, Nasal blockage with occasional discharge for 2 months,Cough with blood mixed sputum for same duration & Fever for 20 days . about 3 months back he developed painful red eye and swelling of right eye with thicky discharge. Discharge was through out the day and more at morning after awaking from sleep, which was purulent and non foul smelling. Gradually there was involved of both eyes and for last 2 days his vision became severely impaired that he could not see anything.

Patient also complaints of Nasal blockage with occasionally watery nasal discharge for last 2 months. For this complaints he admitted in ENT dept. of MMCH. Patient also stated that he has been suffering from cough for 3 months. It was initially dry and then became productive which was scanty in amount ,serous in nature and not foul smelled . For last 15 days he also notice that his sputum is occaisonally blood stained. There is no history of chest pain, dyspnoea, vomiting, haematemesis or bleeding from any other site.

He also complaints of High grade , intermittent Fever for last 15 days which appeared almost every day and remain for about 1 hour, and subsided spontaneously. Highest recorded temperature was 103 F and fever is not associated with chills-rigor. Patient also complaints of throat pain anf voice change for last 15 days, and difficulty in chewing for last 9 days, on query patient also mention that there is deviation of angle of mouth towards right side.

There is no history of nasal regurgitation, dysphagia or emotional lability. His bowel and bladder is normal. He also complaints of anorexia and significant weight loss during the course of the illness. There is no history of jaundice, oral ulcer, skin rash, joint pain or swelling. On general examination patient is ill looking, co-operative, mildly anaemic, non icteric. Eyes are red, swollen and there is scleral injection , corneal Haziness of both eyes. There is depression of nasal bridge, and crusting on both nasal cavities. There is no cyanosis, clubbing, koilonychias, leuconychia. Oedema, dehydration absent.

Provisional Diagnosis

Vasculitis Syndrome possibly due to Small vessel vasculitis (Wegeners granulomatosis)

Differential Diagnosis

Systemic Fungal infection (Histoplasmosis)Disseminated TuberculosisSarcoidosis

InvestigationsCBC:- (20/10/2015)Hb- 9.8 gm/dlMCV- 84 flMCHC- 27 .4 g/dlESR- 115 mm in 1 st hrTC- WBC count- 16700/ cu mm Neutrophils- 12800 / cu mm (79%) Eosinophil 0.7 % (normal)Platelet count- 579000/cu mm.

Urine R/EColour- StrawAlbumin- TracePus cell- 0-3 / HPFRBC- occasionalNo Cast

S. Creatinine- 0.8 mg/dlSGPT- 30 U/LRBS- 7.6 mmol/LSputum for AFB- negativeSputum for Fungal Hyphae- report pending

Chest X ray P/A view: Multiple cavitary lesion involving both lung field more marked in upper and midd zone .

ECG: Normal

Second Line Investigation:

c ANCA , p ANCA : Result Pending.RA FactorBiopsy from nasal mucosa: Yet not done.Renal Biopsy

Confirm Diagnosis???

Thank you