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Unraveling a Rare Presentation of an Uncommon Disease: A Pyrexia Case Report

Explore a detailed case report of a young female's journey through symptoms, diagnosis, and treatment for a complex condition involving Takayasu arteritis with pulmonary artery involvement. Witness the challenges faced and expert interventions provided.

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Unraveling a Rare Presentation of an Uncommon Disease: A Pyrexia Case Report

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  1. Case Report for Delhi-API 2016 Dept. of Medicine LHMC and associated Hospitals A case of Pyrexia of Unknown Origin :A rare presentation of not so uncommon disease Presenter: Dr Udit Aggarwal PG Medicine LHMC

  2. History • SB/25yr/F/non smoker • Presented to a pvt. hospital in Delhi (May 2009) • with :C/O Dry cough X 1 .5 year • Low grade fever X 1 yr • Dyspnoea on exertion (NYHA class II-III ) X 3months • One episode of Fever with sore throat, Lt earache and tinnitus in Feb. 2008 • She was Rx with oral antibiotics but developed decreased hearing in Lt yr • ENT surgeons: severe sensory neural hearing loss in Lt ear.

  3. History contd…. • H/o palpitation on exertion+ • h/o skin allergy in the form of Hives formation + • No h/o joint pain/photosensitivity/alopecia • No H/o bladder or bowel complaint • No significant appetite or weight loss • Menstrual history: regular 4-5 days/28-30 days cycle

  4. Examination( pvt hosp) • General physical examination: Pallor+, no nodes, no edema, no rash, joints normal). • Vitals: PR: 104/min • BP: 100/70 mmHg Rt arm supine • RR: 16 /min abd-thoracic • Chest examination: B/L VBS, • CVS: soft systolic murmur ? In Pulm. Area (hemic) • Abdominal : No hepatosplenomegaly • CNS examination: WNL.

  5. Lab Investigation • Hb: 9.7gm/dl, TLC: 9,900/cmm, (N56%,L22%,M8%,E4%), ESR 105mm/1st hour, Platelets: 440,000/cmm, MCV: 71 (76-100fl), MCH 24.4pg (27-32), MCHC 34.3 gm/dl. • Peripheral smear – microcytic hypochromic anemia. • Blood sugar – fasting and PP normal. • Renal function tests: normal, • OT/PT 23/10 U/L, S bilirubin – 0.8 mg/dl, S. Albumen 3.9g/dl, globulin: 4.6g/dl, • Lipid profile normal, thyroid function normal.

  6. Lab Investigation contd.. • UGI Endoscopy normal. • ENT examination: Upper airway WNL, Lt yr sensory neural hearing loss-severe. • Echocardiograhy – EF 60%, mild jerky motion of IVS, mild TR, PASP 31, mild dilatation of main pulmonary artery, mild posterior pericardial effusion. • Serum electrophoresis – generalized increase in all globulin fraction, • Serum Iron studies: S iron: 0.03(.04-0.13)mg/dl. S Ferritin 95 (30-300 ng/ml), TIBC :225 (260-390). • Hb Electrophoresis: HbA2 : 3.6( ?N <3.4%) • Sputum AFB negative three times, • Serum ACE 26 U/L. PFT – normal study ( FVC 3.39L, FEV1 2.99L).

  7. CXR - prominent right hilum, lung field normal.

  8. CECT chest & other radio- imaging • CECT chest – subcentimetric mediastinal nodes (not significant). ? Mild Pulm A dilatation. Aorta and Other vessels normal • US abdomen and pelvis – normal. • MRI :brain normal.

  9. Provisional Diagnosis and treatment( in pvt hosp) • Chronic cough ? Allergic • Microcytic Hypochromic Anemia with Iron Deficiency Anemia • Sensory Neural Hearing Loss ? Cause/ ? post infective • With Idiopathic Pulmonary artery dilatation (asked to follow yearly)

  10. Follow-up Visit • Progressive dyspnoea and dry cough X 1.5 yr • F.U.O X 1yr( >3 wks, >38.3 C temp, 3 OPD visit) • Raised globulin and IgG levels • ESR Raised • Hematology opinion to R/o Multiple Myeloma/paraproteinemia • ENT surgeons: SMHL lt yr, R/o autoimmune dis. Referred to medicine

  11. Hematologist opinion • Serum Electrophoresis repeated: normal polyclonal gammaglobulin, No M spike. • Bone marrow Exam: Normal Hematopoietic cells with normal myeloid and lymphoid series • No evidence of blast or granuloma • Bone marrow aspirate: PCR for M tuberculosis +ve • Started on ATT-HRZE: No improvement after 4 months • Dyspnoea progressive • Fever + intermittent • ESR raised Referred to Medicine dept for expert opinion and further management( MDR-TB)

  12. 2nd opinion: Admitted and Re-evaluated in medicine Dept. • F.U.O: Low grade fever with Evening rise of temp, since June 2008( 1yr) • Dyspnoea on exertion, class II-III • Dry Cough • Generalised malaise • On ATT-HRZE for past for 4 months on basis of PCR +ve for MTB • ANA/RF/ANCA/dsDNA : all negative • C3 levels: normal

  13. On Examination: PR:96/min Right Radial and Brachial artery pulses appeared feeble compared to left, other pulses were well felt except for Rt carotid There was a carotid bruit+, over Rt Carotid Artery BP: Rt UL: 100/70, Lt UL: 120/80 Rt LL: 140/100 and Lt LL: 136/90 mmHg • Temp: 99 degree F • RR;18/min • No Abdominal / renal bruit heard

  14. Re-evaluation/ Investigaton • Hb: 9 g/dl • TLC: 9600/cumm • DLC: P72L19M08 • Platelet count: 4.97 lacs/cumm • ESR: 125 1st hr • CRP: 104.3 mg/L(<5) • P/S: hypochromia and anisocytosis. Microcytes+ • Iron studies: o.02(.04-0.13 mg/dl), TIBC: 0.38 (0.26-0.39 mg/dl) • Bil T: 0.6 mg/dl • Prot T/Alb: 7.2/3.7 /3.5 g/dl • OT/PT: 18/19 • SAP: 368U/l (100-290) • CECT chest and Abdomen with CT angio was planned .

  15. CT Angiography

  16. MR angio

  17. MR angio

  18. Final Diagnosis • Diffuse Takayasu arteritis ( Type III) with pulmonary artery involvement(Type IV). • With sensory neural hearing loss ( ?post infective)

  19. Follow up and treatment • Pt was treated with tab prednisolone 1mg/kg/day for 6 weeks followed by tapering dose of wysolone • ATT maintenance phase continued for 2 months as she was already on it. • Pts fever and cough has disappeared • Her ESR came down from 105 mm to 40 mm in 1 month • Her CRP came down from 104.3 to 9.90 mg/dl(0-6)

  20. THANKYOU Acknowledgement: Dr. Randeep Guleria/ Dr Ghanshyam Pangtey

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