• Mr.rajan 38 yr old smoker• C/O swelling all over the body- past 4 months

5months back-he developed1. Cough-productive,scant mucoid sputum 2. chest pain-diffuse pricking increases with cough3. Breathlessness-Class iii(NO orthopnoea/PND)• No chest pain/palpitation• No haemoptysis/fever• No oliguria/dysuria/haematuria

On Examination

• Pt conscious oriented • Afebrile• No pallor /icterus / cyanosis/clubbing• B/L pitting pedal edema• Not dyspnoeic• No generalised lymphadenopathy• JVP not elevated• PR 86/min BP 110/70mm Hg

INFECTIVE CAUSES• mycobacterial –• Bacterial- staph aureus,klebsiella,psuedomonas,nocardiosis,

anaerobes• Fungal- aspergillus,blastomycosis,coccioidomycosis,

histoplasmosis• Septic emboli• Lung abscess

DIFFERENTIALSFOR MULTIPLE CAVITARY LESIONS

TRAUMA • Pulmonary contusion• Septic emboli• Lung abscessGRANULOMATOUS, INFLAMMATORY DISORDERS• Necrotizing sarcoid granulomatosis/lung• Wegeners granulomatosisCOLLAGEN VASCULAR DISORDERS• Polyarteritis nodosa• Rheumatoid lung diseaseVASCULAR DISORDERS• Pulmonary infarction with cavitationNEOPLASTIC DISORDERS• Lymphomas• Metastatic lung disease• carcinoma of lung

LOOKING AT CAVITIES• What is the anatomic distribution?

– Is It single or multiple? – If multiple , are they focally located or diffusely seen in

multiple lobes of both lungs?

• Is are the characteristics of the cavity?– Is the cavity wall thick or thin?– Is the interior lining nodular, shaggy, or smooth?

• Are there any other associated radiographic abnormalities?

INVESTIGATIONS

• Urine routine-protein 2+, 3-5 RBCs• TC 13800• DC P66 L 32 E2• ESR 56mm/hr• Platlets 1,45,000• Hb 12.8 g• Urea -17 mg• Creatinine 0.8 mg

• HIV – NR• SPUTUM AFB negative• Sputum c/s no growth• RF – neg• ANA – Neg• CRP elevated• ENT OPINION : Polyp from Lt middle

meatus, VLS-normal

FNAC OF A NODULE(CT GUIDED)

POSSIBILITIES

• 1.WEGENER’S GRANULOMATOSIS• 2.PULMONARY LANGERHANS CELL

HISTIOCYTOSIS

P-ANCA … POSITIVE

Approximately 90% of patients with active Wegener’sgranulomatosis have a positive antiproteinase-3 ANCA.

A small percentage of patients with Wegener’s granulomatosis may have antimyeloperoxidase rather than antiproteinase-3 antibodies.

Points in favour

• Renal involvement• P-ANCA positivity• Radiolgical features• Paranasal sinus involvement

Wegener granulomatosisWegener granulomatosis• Granulomatous, necrotizing, small & medium vessel vasculitis

• Any age (40-55 yrs.)

• Frequency of 3 per 100,000 persons

TRIAD RENAL-Microscopic hematuria, proteinuria, and rapidly progressive RF UPPER AIRWAYS- recurrent / nonresolving sinusitis, epistaxis, nasal

septal perforation (saddle nose deformity) LOWER AIRWAY- subglottic stenosis. Single or multiple

cavitating/noncavitating Pulmonary nodules or diffuse alveolar hemorrhage

Wegener granulomatosisWegener granulomatosis• Skin rash, migratory arthritisSkin rash, migratory arthritis

• Ocular involvement (scleritis, corneal ulceration,orbital dis.)Ocular involvement (scleritis, corneal ulceration,orbital dis.)

• Mononeuritis multiplex or CNS involvement with or without Mononeuritis multiplex or CNS involvement with or without pachymeningitispachymeningitis

• ANCA (180pt., 96%with severe dis.,83%with limited dis.)ANCA (180pt., 96%with severe dis.,83%with limited dis.)

• Lung biopsy yield 91% of casesLung biopsy yield 91% of cases

• Upper airway biopsy yield only 21%of casesUpper airway biopsy yield only 21%of cases

• Renal biopsy:focal, segmental necrotizing, crescentic Renal biopsy:focal, segmental necrotizing, crescentic glomerulonephritis with few to no immune complexes glomerulonephritis with few to no immune complexes

ANCA

• ANCA plays a central role• Antibodies directed against certain cytoplasmic

proteins in neutrophils and monocytes• Two major categories based on IF– C & P ANCA• Present in high percentage in systemic vasculitis

Kallenberg CGM et al. (2006) Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides Nat Clin Pract Rheumatol 2: 661–670 doi:10.1038/ncprheum0355

Figure 1 Cytoplasmic components of ethanol-fixed neutrophils, stained by indirect immunofluorescence, in a serum sample from a patient with active

Wegener's granulomatosis and antineutrophil cytoplasmic autoantibodies to proteinase 3

RENO-PULMONARY SYNDROMES1.ANTI-GBM ANTIBODIES:

Goodpasture’s syndrome2.ANCA-POSITIVE VASCULITIS• Wegener’s granulomatosis• Microscopic polyangiitis• Churg–Strauss syndrome3.ANCA-NEGATIVE VASCULITIS• Henoch–Schönlein purpura• Mixed cryoglobulinaemia• Behçet’s disease• IgA nephropathy4.INFECTIONS

5.DRUGS• Propylthiouracil• D-Penicillamine• Hydralazine• Allopurinol6.RHEUMATIC DISEASES (immune complexes)• Systemic lupus erythematosus• Scleroderma• Polymyositis• Rheumatoid arthritis• Mixed collagen vascular disease