CME INDIA Case Presentation by Dr Nishith Kumar, Pulmonologist, Ranchi.

CME INDIA Case Study

CT Thorax

CT Thorax is suggestive of nodular infiltrates in Rt Upper Lobe & Rt Middle Lobe with mediastinal lymphadenopathy

CME INDIA Discussions

Dr Sanjeev Yadav, Heamatologist, SGPGI, Lucknow:

• Rule out non-Hodgkin’s lymphoma, CT guided Biopsy/ IHC, PET CT & Bone marrow examination needed. Any past H/O TB, fungal pneumonia, Sr Galactomannan/ Procalcitonin?

Dr Anand Malani MD, Sangli:

D/D

• Disseminated TB
• Lymphoma
• Metastatic lesions
• Less likely Sarcoidosis

Supraclavicular node biopsy

Or Endoscopic ultrasound guided mediastinal node biopsy. Wouldn’t transoesophageal be easier and safer? Endoscopic USG is a very promising tool. We use it very commonly and almost always successful. No Anaesthesia needed. And again, supraclavicular node biopsy was also an easy option. Just my thoughts

Dr Nishith Kumar, Ranchi:

• Hilar Lymphadenopathy with parenchymal lesions in form of micronodules with a peri lymphatic distribution is hallmark of Sarcoidosis.
• Tuberculosis remains an important DD in India in such cases however presence of non-necrotic LN on radiology makes the diagnosis of Tuberculosis less likely.
• In view of presence of parenchymal lesion along with mediastinal lymphadenopathy there’s a definite indication for Bronchoalveolar lavage/sputum sampling.
• Whenever Right Paratracheal LN or Subcarinal LN is involved & size is more than 10mm Blind TBNA achieves fair amount of success in hitting the target & generally gets the job done in expert hand. EBUS/EUS no doubt is the best modality but is costly (5.5k vs >30k) & is not available everywhere.
• So, to conclude Individually, EBUS-TBNA has the highest diagnostic yield in sarcoidosis, but it should be combined with TBLB for the optimal yield. The diagnostic yield of conventional TBNA + Endobronchial biopsy and Transbronchial Lung Biopsy is similar to EBUS-TBNA plus TBLB.

Did Bronchoscopic Transbronchial Needle Aspiration from Subcarinal @ Right Paratracheal Lymph nodes

Bronchoscopic TBNA from Subcarinal LN

TBNA Cytology report – Non necrotising granuloma, no AFB seen

Dr Nishith Kumar Pulmonologist Ranchi:

This was basically a case of Sarcoidosis. DD includes (as rightly mentioned by Dr Sanjeev Yadav) Lymphoma, Tuberculosis & Sarcoidosis.

Dr K N Manohar, Bengaluru:

HIV?

Dr Nishith Kumar:

Negative

Dr Sanjeev Yadav:

Any other test to confirm Sarcoidosis?

Vit D levels

Dr Nishith Kumar, Ranchi:

• Gallium scans
• PET Scan do have certain role but have low sensitivity & specificity.
• Elevated alk phos, Hypercalcemia & Hypercalciuria can be present in small percentage of patients. Kveim test is no longer routinely done.

Dr Ranjeet Kumar, Ramgarh:

What are the +ve findings in Xray?

Dr Nishith Kumar, Ranchi:

• Tell-tale signs of Mediastinal Lymphadenopathy can be seen on CXR
• There’s something called Scadding criteria…
• On PA chest radiographs, pulmonary sarcoidosis can be classified into five stages, stage 0-IV

CME INDIA Learning Points

(By Dr Nishith Kumar, Pulmonologist, Ranchi)

Signs and symptoms

• The presentation in sarcoidosis varies with the extent and severity of organ involvement.
• Many patients may remain.
• Asymptomatic & the abnormality may be incidentally detected on chest imaging (Approximately 5% of cases).
• Symptomatic patients may present with:
  • Systemic complaints like fever, anorexia and
  • Pulmonary complaints like dyspnea on exertion, cough & chest pain.
  • Löfgren syndrome (fever, bilateral hilar lymphadenopathy, and polyarthralgias) is another common finding.
• Dermatologic manifestations may include the following:
  • Erythema nodosum
  • A lower-extremity panniculitis with painful, erythematous nodules (often with Löfgren syndrome)
  • Lupus pernio (the most specific associated cutaneous lesion)
  • Violaceous rash on the cheeks or nose (common)
  • Maculopapular plaques (uncommon)
  • Imaging studies plays important role in workup for sarcoidosis.
• Chest radiography: Central to the evaluation
  • High-resolution CECT scanning of the chest: May be helpful; identifies active alveolitis versus fibrosis, along with assessment of mediastinal lymphadenopathy and findings correlate with biopsy yield.
  • PET Scan & Gallium scans: Used infrequently; has low sensitivity and specificity.
• Staging of sarcoidosis based on chest radiology is as follows:
  • Stage 0: Normal chest radiographic findings
  • Stage I: Bilateral hilar lymphadenopathy
  • Stage II: Bilateral hilar lymphadenopathy and infiltrates
  • Stage III: Infiltrates alone
  • Stage IV: Fibrosis
• Routine laboratory evaluation is often unrevealing, but possible abnormalities include the following:
  • Hypercalcemia (about 10-13% of patients)
  • Hypercalciuria (about a third of patients)
  • Elevated alkaline phosphatase level
  • Elevated angiotensin-converting enzyme (ACE) levels
  • Negative Mantoux test
• Diagnosis requires biopsy in most cases. Endobronchial biopsy via bronchoscopy is often done. The central histologic finding is the presence of noncaseating granulomas with ZN stains negative for mycobacteria.
• Management
  • Patients with stage I sarcoidosis often require only occasional treatment with NSAIDs.
  • For Symptomatic Stage II & III patients & patients having extrapulmonary sarcoidosis involving critical organs such as the heart, liver, eyes, kidneys, or central nervous system, corticosteroid therapy is indicated.
  • Common indications for steroid sparing agents include the following:
    1. Steroid-resistant disease
    2. Intolerable adverse effects of steroids
    3. Commonly used steroid sparing agents includes:
    4. Methotrexate,
    5. Azathioprine,
    6. Cyclosporine, Infliximab, Mycophenolate etc.

CME INDIA Tail Piece

• Sarcoidosis is a multisystem disorder of unknown etiology that mostly affect young adults.
• It presents with noncaseating granulomas in various organs.
• Characteristically it presents with bilateral hilar lymphadenopathy and reticular opacities in the lungs.
• Other major involved sites include skin, eyes, and joints, although it can express to a variable degree in the musculoskeletal system, reticuloendothelial system, exocrine glands, heart, kidney, and central nervous system.
• D/D
  1. Tuberculosis
  2. Cat scratch disease
  3. Lung cancer
  4. Lymphoma
  5. Occupational lung disease
  6. Fungal infection
• Management
  • Stage I: Pulmonary sarcoidosis is often asymptomatic, non-progressive disease and requires no treatment
  • Majority of patients undergo spontaneous remission.
  • Close monitoring of symptoms, chest radiograph, and pulmonary function is continued at three to six-month intervals should be considered in asymptomatic patients.
• Patients with pulmonary sarcoidosis causing worsening symptoms, stage II-III radiographic findings should:
  1. Oral glucocorticoids at 0.3 to 0.6 mg/kg for 4 to 6 weeks.
  2. If there is no improvement in symptoms, radiographic abnormalities, and pulmonary function tests, steroids may be continued for an additional four to six weeks.
  3. Maintenance steroids are not needed, steroid tapering to a dose of 0.25 to 0.5mg/kg (usually 10 to 20 mg) per day, should be considered over a period of at least six to eight months.
  4. Methotrexate, azathioprine, infliximab, leflunomide, and antimalarial agents may be considered as steroid-sparing agents in patients who are unable to tolerate steroids.
• Prognosis
  • Asymptomatic patients do not require treatment and often remain stable for many years. However, those who develop symptomatic lung or extrapulmonary disease tend to have a guarded prognosis.
  • Relapse of symptoms is common and many patients with advanced disease develop dyspnea and pulmonary hypertension. The overall mortality rate for untreated patients is about 5%.

(Reference: Landi C, Carleo A, Cillis G, Rottoli P. Sarcoidosis: proteomics and new perspectives for improving personalized medicine. Expert Rev Proteomics. 2018 Oct;15(10):829-835.)

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