15-20% of microscopic polyangiitis are ANCA (false) negative with ESR upto 130-150 mm / I hour Westergren, going on to renal failure. I maintained such a patient (female, no renal biopsy, Hb falling to 5g /100 ml, MCV 72, normal ferrokinetics, few acanthocytes, no diabetes mellitus, DS DNA negative, ANA positive speckled pattern, with normal renal size on USG but with renal hypertension and mild ACE positivity) , a female in her seventh decade for 8 years (2004 to 2012) with a series of medications, including methlyprednisolone and other immunomodulatory agents , one blood transfusion, with referral to a renal unit (Max Hospital, Saket, New Delhi) with no change in therapy except for an admonition not to visit me (not a qualified neurologist) again. She is still alive. I qualified in 1977 when Nephrologists in India were few and far between.

There is an unseemly turf battle between so called Nephrologists and non Nephrologists (Internists), who with a comprehensive grasp of medicine are able to manage such patients early on and prevent ESRD. Very recently, a disgruntled Neurologist mocked my pretense of working as a Neurologist in lacunar infarcts in the columns of ResearchGate. I also stand at the cusp of being the leading authority in PFO closure as well as misuse of magnesium in the ICU, to the chagrin of many cardiologists and intensivists (Expert Rev Neurother, 2009, 2010 Open Access).

The jealousy between specialists and competent non specialists is at the cost of welfare of such patients.

I have maintained another ANCA negative patient for 3 decades, discharged from a tertiary care hospital in Dubai, UAE, as a terminal case with imminent death in a week or so in 1989, and currently maintaining good health in Karachi, Pakistan.

Another ANCA negative patient survived under my care for 10 years, after being extensively investigated at the Renal Unit at Manchester, Stockton, UK for idiopathic hypercalcemia (Nephron, Open Access, 1996).

ORCID ID: 0000 0002 6770 5916

I agree that there can be ANCA or MPO negative MPA. We have had AAV patients with reversible systolic cardiac dysfunction in a few patients. Cardiac involvement though rare has been seen by us in a handful of patients.

Thanks for your replies. I have been searching the literature on this topic and according to that, it seems there are 5-10% ANCA-negative MPA cases. ANCA may become positive in the course of the disease in some patients. Hence biopsy remains the gold standard means of diagnosis.

The case I encountered was a young man who presented with hypertensive emergency and pulmonary symptoms however did not have evidence of kidney involvement initially. A case report presentation with biopsy results will be available soon.

In my opinion and experience, ESR is the most fail-safe and most-neglected feature.

I have a GBS with paraplegia and p-ANCA-positive but no renal involvement.

Article Pauci-immune glomerulonephritis: Does negativity of anti-neu...

Renal biopsy is not possible in all cases; my current ANCA-positive patient has donated a kidney 5 years ago. We worry whether renal biopsy will add to academic knowledge or patient management. Sometimes a toss-up or clinical compromise has to be settled for.

Aman, thank you for the reference of ANCA-negative PIGMN. What are your experiences of systemic extra-renal involvement in such cases at PGI Chandigarh, published or non-published yet?

Dr Gupta ,

I am sharing our published experience. You may choose the relevant

1)Article Successful treatment outcomes in pregnant patients with ANCA...

2)Article Clinical features and long-term outcomes of 105 granulomatos...

3) Article Impact of renal involvement on survival in ANCA-associated vasculitis

4Article Histopathological classification of pauci-immune glomerulone...

5 ) Article Uncommon presentations of primary systemic necrotizing vascu...

Dr Aman,

Thanks. Will review all attachments.

Dr Aman Sharma these are great articles, thank you for sharing!

Poster Pursuit of A Clue

Dear Drs Aman Sharma and Vinod Kumar Gupta , I would like to share with you my recent case report on a patient with ANCA-negative MPA. I would appreciate your input and recommendations on this case. Thank you so very much.