CME INDIA Case Presentation by Dr Ashutosh Chandan Dubey (Dr Strange), DNB (Med), Renukut, UP.

CME INDIA Case Study:

54-year-old female with productive cough, multiple bouts of haemoptysis, chest pain and SOB for past 10 years.

What it looks like:

Dr Ravishankar Dwivedi Dermatologist, Ranchi: Lichenoid rash/Oral and topical steroids/ cyclosporin/ oral retinoid treatment options.

Now see these pics:

Dr Amit Kumar, Dermatologist, Ranchi: Seeing the previous pics can go with lichenoid dermatitis. However, the later pics are clear psoriatic lesions. So, I will disagree now and diagnosis is Psoriasis vulgaris. Oral steroids should not be given. Rest all treatment modalities as mentioned by Dwivedi Sir can be given. I would however like to plan for methotrexate or Apremilast in this case.

Dr R. Dwivedi: Biopsy/ Dermascopy will give definite diagnosis. Clinically still in favor or LP. D/d Psoriasis/ SCLE.

Pattern looks more like drug induced lichenoid.

Final Diagnosis:

• Wegners granulomatosis with lichen planus, mononeuritis multiplex, Guttate psoriasis and and Cushing syndrome.

Now see the details:

• A 54yr. old lady has come with productive cough, multiple bouts of haemoptysis, chest pain and SOB for past 10 yrs. which has progressed over last 2 yrs.

• She also has black pigmented pruritic rashes over left anterior thigh and papulo-nodular diffuse rashes over all 4 limbs, back and abdomen. The rashes are more prominent during night.
• She has been suffering from Wagner’s Granulomatosis and undergoing treatment. There had been multiple episodes of relapse and remission.
• Initially it started with recurrent allergic rhinitis, Acute otitis media and headache following sinusitis. She was on anti-histaminic for quite long time until in 2012 when she developed B/L pedal oedema and was thoroughly investigated.
• Renal biopsy was done showing FSGN without any immune deposit and positive for C-ANCA.

• CT thorax was normal but gradually she developed cough, chest pain, SOB and finally Haemoptysis. Serial CT thorax were done showing multiple cavitary lesions accompanied by fungal infection.

• She was started on Steroid and cyclophosphamide and with multiple relapses and disease progression was put on Rituximab maintenance therapy.
• Gradually she developed paraesthesia and distal weakness of limbs which is asymmetric and limited to Rt sided only. Reduced lateral corticospinal tract sensation and power of 4/5 in distal joints on rt side. Posterior column sensation is intact.

• Now she has features of cushing syndrome with moon like face, hirsutism, multiple bone pain – Xray and Dexa scan showing osteopenia.

CME INDIA Learning Points

• Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology.
•  Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.
• GPA is part of a larger group of vasculitic syndromes.
• They all feature an autoimmune attack by an abnormal type of circulating antibody called antineutrophil cytoplasmic antibodies (ANCAs). The specific blood test used to diagnose and monitor granulomatosis with polyangiitis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.
• ANCA is 66% sensitive and 98% specific for GPA and is present in 80-90% of the patients with active multisystemic disease. There are some cases where ANCA is negative. Cytoplasmic-ANCA (c-ANCA) with autoantibodies directed against proteinase 3 antibodies (PR3) is seen in 80-90% of the cases with GPA, and the remaining are perinuclear-ANCA (p-ANCA) directed against myeloperoxidase antibodies.
• The diagnosis of granulomatosis with polyangiitis is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of the condition.
• Granulomatosis with polyangiitis commonly involves a triad of:
  1. Upper respiratory tract (sinusitis, crusting rhinitis, saddle nose deformity, otitis media, mastoiditis, hearing loss), and lower respiratory tract (lung nodules, alveolar hemorrhage).
  2. Systemic vasculitis.
  3. Kidney involvement (glomerulonephritis).
• The ACR diagnostic criteria include: a) urinary sediment showing red blood cell casts or more than five red blood cells per high power field, b) abnormal findings on chest radiograph, c) oral ulcer or nasal discharge, and d) granulomatous inflammation on biopsy. The presence of two or more out of the above mentioned four criteria was associated with a 92% specificity and 88% sensitivity.
• The ELK (E stands for ears, nose, and throat or upper respiratory tract, L for lung and K for kidney) proposed by DeRemee utilizes ANCA to diagnose. Per these criteria, any typical manifestation involving the ELK along with positive c-ANCA or typical histopathological finding qualifies for a diagnosis of GPA.

CME INDIA Tail Piece

• The name “Wegener’s granulomatosis” is no longer used because the German physician Friedrich Wegener, the disease’s former namesake, was a member of the Nazi party and allegedly linked to Nazi war crimes.
• Granulomatosis with polyangiitis usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of the condition is not known.

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