Photo Quiz - December 2018

A 55-year old lady from Jhapa, Nepal presented with fever and dry cough for 2 months. She had a history of epistaxis and haemoptysis on 2 occasions. High resolution computed tomography (HRCT) revealed multiple nodular lesion in both lungs. Sputum culture was positive for methicillin–sensitive Staphylococcus aureus (elsewhere). She was being treated for pneumonia with broad spectrum antibiotics (piperacillin-tazobactam, meropenem, linezolid) in Nepal and Siliguri without improvement. She subsequently sought Infectious Diseases (ID) consultation and was found to have fever (1020F), dry cough, redness in eyes and multiple oral ulcers. Total leucocyte count was 20240/μl of blood, platelet count reached to 223,000/μl of blood with raised CRP (207.8 mg/L) and elevated ESR(67mm/hr). Her repeat CT chest with contrast revealed multiple nodular lesion which has increased in size then previous lesion (Figure1) with enlarged nodes in bilateral hilar region. CT also revealed diffuse infarction of spleen. Contrast paranasal sinuses (PNS) revealed no significant lesion. Bronchoalveolar lavage (BAL) was performed which showed that gram stain was negative for Gram positive cocci (GPC) and Gram negative bacilli (GNB). Cultures were negative and multiplex PCR was positive for human-metapneumovirus. Urine routine showed pus cells (30-40/hpf) and numerous RBCs. But on enquiry, patient had no symptoms suggestive of urinary tract infection. Blood cultures were negative.

Figure 1: CT Chest

What is your diagnosis? View Answer

Answer

In view of upper respiratory tract symptoms, haematuria, lung nodules, splenic infarction, BAL negative for GPC or GNB. c-ANCA (PR-3) was performed which was positive suggestive of granulomatosis with polyangitis.

Differential Diagnosis of Lung Nodules

Bacterial

  • Staphylococcus aureus
  • Klebsiella
  • Pseudomonas
  • Legionella
  • M Tuberculosis
  • Nontuberculous mycobacteria
  • Nocardia
  • Burkholderia pseudomallei
  • Burkholderia cepacia
  • Rhodococcus
  • Thoracic Actinomycosis

Fungal

  • Aspergillus
  • Cryptococcus
  • Mucor
  • Histoplasmosis
  • Fusarium
  • Scedosporium
  • Coccidioidomycosis

Parasitic

  • Paragonimus westermani
  • Hyadatid cyst

Viral

  • CMV

Non-infectious causes

  • Bronchogenic carcinoma
  • Granulomatosis with polyangitis
  • Metastasis

Granulomatosis with polyangitis (Wegener’s) is a small-vessel vasculitis causing upper respiratory symptoms like epistaxis, perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis. Lower respiratory tract symptoms include hemoptysis, cough, dyspnea and renal symptoms like haematuria and red cell casts. It is the triad of focal necrotizing vasculitis, necrotizing granulomas in the lung and upper airway and necrotizing glomerulonephritis. Chest X-ray usually shows large nodular densities and get confirmed by positive of c-ANCA/ PR3-ANCA. Treatment of choice is pulse therapy with steroids, cyclophosphamide.

Final diagnosis: Small-vessel vasculitis- granulomatosis with polyangitis (Wegener’s)

Case provided by: Dr Ranjit Sah (ID Fellow), Sony Chawla (clinical research coordinator), Dr Sandeep Kumar Mittal (Pulmonology), Dr Rajiva Gupta (Rheumatology), Dr. Lucky Sharma (Rheumatology), Dr Monica (Radiology), Dr Neha Gupta