After starting the patient on treatment, monitoring of disease activity and response to treatment can be assessed by the following clinical and lab parameters.

CLINICAL

•      Existing lesions of vasculitis starts healing which is made out clinically by fading out of maculo-papular dusky red erythematous lesion leaving a hemosiderin induced post-inflammatory hyperpigmentation.

•      No new lesions of vasculitis should be encountered.

LAB PARAMETERS

•      Fall in the titre of markers of acute inflammation like ESR & CRP.

•      Fall in the titre of ANA, dsDNA and ANCA.

•      Reduction in the Brimingham Vasculitis Activity Score (BVAS)

Patients have to be monitored with repeated blood tests, once in 3 months to watch for markers of further progression of disease in the form of appearance of neoantigens and antibody like dsDNA becoming positive later in the course of the disease.

In our study, in majority of patients (53%), the etiology was idiopathic. Vasculitis in the background of SLE contributed to 12 patients (16%). ANA titre and other antibodies were monitored more diligently in those patients.

In our study, one patient who was recalcitrant to treatment progressed to ANCA positivity during the course of illness and developed endophthalmitis and interstitial lung disease.

We also encountered a young female patient of age 25 years who presented with seizures and malignant hypertension with spontaneous non-healing ulceration in both legs. Her ANA and ANCA profile initially was negative and despite treatment, her ulcers didn’t heal. On regular follow up of the patient, her ANA and ANCA became positive within a span of 6 months and were subsequently treated with steroids for Polyarteritis Nodosa (Figure 1).

Similarly, a middle aged woman presented with recurrent episodes of persistent urticarial wheals over both extremities and trunk. Vasculitis workup was done. ANA was negative. CRP was found to be elevated (Figure 2).

As recurrent Urticarial Vasculitis may be a presenting feature of SLE and Sjogren’s syndrome [4], patients with this kind of presentation should be kept on a long term vigil, so as to detect evolving SLE and Sjogren’s earliest possible.

Earnest efforts should be made to reach a definite diagnosis in these types of patients, as majority of them have features suggestive of undifferentiated connective tissue disorders and overlap syndromes. This needs a comprehensive approach towards understanding of evolution of the disease process with respect to chronology of appearance of serological markers viz., varying Extractable Nuclear Antigens, the corresponding Auto-antibodies and the symptom complex resulting out of them. Predicting prognosis and choosing therapeutic options in these cases will by and large depend on the extent of organ involvement.

CONCLUSION

Benign Idiopathic CSVV is a self-limiting entity, settles down in 6 months to 1 year of treatment, provided patient does not develop any other surrogate markers like ANA, dsDNA and ANCA during the course of illness.

Patient of vasculitis who remains ANCA negative at the point of commencement of treatment, can become ANCA positive at any point of time, thereby signaling ominous sign, as with ANCA positivity, vasculitis starts involving medium sized and large sized vessels thereby leading to loss of function of vital organs, which could at times be fatal, as it can lead to conditions like Panophthalmitis, Endophthalmitis and Interstitial Lung Disease. If C-ANCA and P-ANCA coexist in a patient, drug-induced vasculitis should be suspected.

It was found out in our study that 80% of patients with CSVV responded well to systemic steroids, the simplest and safest immunosuppressant. Also it was inferred, that maintenance of patients with more than one immunosuppressant at a particular point of time is not advisable as the risk of complications like opportunistic infections, pancytopenias and avascular necrosis of femur, is on the higher side.

CSVV in ANA positivity background needs investigations and diligent follow up as it may develop into full blown SLE and hence patients have to be offered a guarded prognosis [5]. In tropical countries like India, Tuberculosis and SLE mimic each other closely in the early stages of evolution [6,7]. Hence sufficient precaution and exhaustive work up is always mandatory to rule out Tuberculosis before starting the patient on immunosuppressant.