Cocaine and ANCA Associated Vasculitis-like Syndromes – a Case Series

Sujith Subesinghe¹, Sander van Leuven², Leena Yalakki³, Shirish Sangle (Joint First Author)³ and David P. D'Cruz³, ¹Rheumatology and Lupus, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, United Kingdom, ²Rheumatology, Radboud University, Nijmegen, Netherlands, ³Louise Coote Lupus Unit, Rheumatology and Lupus, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, United Kingdom

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: ANCA, inflammation and vasculitis

Session Information

Date: Monday, November 6, 2017

Title: Vasculitis Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Cocaine is a potent illicit stimulant and may trigger a ÔpseudovasculitisÕ mimicking idiopathic ANCA vasculitis. We describe the clinical and serological manifestations of patients presenting with cocaine pseudovasculitis from the experience of a single tertiary rheumatology centre.

Methods:

The electronic patient record and clinic correspondence of 14 patients presenting consecutively between 2000 and 2017 to GuyÕs and St. ThomasÕ Hospitals London with cocaine pseudovasculitis were analysed. All patients disclosed a history of chronic habitual cocaine use. All patients had blood samples analysed for ANCA antibodies, inflammatory markers, haematology and biochemistry profiles. Eleven patients had CT sinus imaging, 8 had chest CT scans. Ten patients had tissue biopsies (renal, skin or nasal/sinus) during clinical work-up.

Results:

There were 10 male and 4 female patients, median age 39 years (range 25-52 years). The mean duration of cocaine use prior to presentation was 9.6 years (range 6-15). Twelve patients had significant sinus thickening or erosive nasal disease. Other multi-system manifestations included vasculitic rashes, pulmonary lesions and peripheral neuropathy. All patients had positive ANCA titres at presentation, persisting in 8 subjects despite clinical remission and drug cessation. Four patients had unusual ANCA patterns (P-ANCA, PR3 positive) with persistently positive ANCA on repeat assessment. Acute and chronic inflammatory changes were noted in all biopsy samples but no granulomas were visualised in the patients who underwent sinus or nasal biopsy. All patients were managed with corticosteroids +/- methotrexate and co-trimoxazole, 2 patients received cyclophosphamide.

The baseline demographics, laboratory assessments, serological status and clinical manifestations (including histological findings where available) are presented in the table. Four patients had positive urinary screens confirming recent cocaine exposure (patients 11 – 14).

Conclusion:

Cocaine induced vasculitis may be difficult to distinguish from idiopathic ANCA vasculitis. Cocaine induced disease is usually associated with localised rather than multi-system involvement. Advanced erosive nasal septal defects, atypical ANCA patterns, and urinary drug screen may be helpful to identify the cocaine induced pseudovasculitis. Complete drug cessation may negate the need for potent immunosuppressive agents. Early referral to drug rehabilitation services may be helpful.

System specific clinical manifestations

Patient

Sex

M/F

Age

(years)

ESR

(mm/h)

CRP

(mg/L)

Serology

(U/ml)

Creatinine

(_mol/L)

Duration of cocaine use prior to presentation (years)

Joint

Skin

PNS

Pulmonary

lesions

ENT lesions

Tissue histology

C-ANCA + PR3 57.5

Repeat ANCA –

–

None (X-ray)

Mucosal thickening maxillary antra. Nasal septum deviation with large defect within it.

Sinus biopsy: granulation tissue, no evidence of vasculitis.

127

P-ANCA + MPO 22

Repeat ANCA –

–

None (CT)

Mucosal thickening maxillary antrum and ethmoid sinuses, nasal septum deviation and destruction of posterior aspect. Destruction of the nasal columella and upper lip.

Sinus biopsy: fibrosis, acute and chronic inflammation. Necrosis at the surface with deeper, microscopic foci of stromal necrosis. Foci of acute venular inflammation, no fibrinoid necrosis. Polymorphic inflammatory cell infiltrate. No granuloma seen.

C-ANCA + PR3/ MPO Ð

Repeat ANCA –

Unconfirmed

–

None (X-ray)

Mucosal thickening, abnormal soft tissue surrounding middle and lower turbinates with erosion and nasal septal deviation.

Sinus biopsy: ulcerated granulation tissue with dense inflammatory infiltrate with neutrophils, lymphocytes.

Renal biopsy: moderate haematuria. Marked fibro-intimal thickening of medium caliber arteries, no evidence of acute thrombotic microangiopathy. Several foci of hyaline arteriosclerosis. Small focus of interstitial fibrosis. No glomerulonephritis.

P-ANCA + PR3/ MPO Ð

Repeat P-ANCA + PR3/MPO –

–

Solitary nodule and calcified granuloma (CT)

Maxillary antrum mucosal thickening. Thickening of osteomeatal complex.

–

P-ANCA + PR3/MPO Ð

Repeat ANCA –

Unconfirmed

–

Calcification (CT)

Mucosal thickening of ethmoid sinuses. Polyploid thickening of right maxillary sinus. Thickened mucosa of middle and inferior turbinates.

Sinus biopsy: active chronic inflammation with a focus of necrotizing vasculitis, fibrinous necrosis and acute inflammation of a venule. No granuloma.

C-ANCA + PR3 381

Repeat C-ANCA + PR3 8.3

–

Left hilar nodes and lower lobe mass (CT)

Air fluid level with associated mucosal thickening. Nasal septal deviation.

Sinus biopsy: ulceration with fibrin deposition and acute on chronic inflammation with plasma cells, neutrophils and lymphocytes. No granuloma or vasculitis seen.

P-ANCA + PR3/MPO Ð

Repeat C-ANCA + PR3/MPO –

Unconfirmed

–

Multi-lobar consolidation, opacification, nodularity (CT)

Air fluid level in right maxillary antrum. Mucosal thickening of ethmoid air cells. Nasal septal deviation.

–

C-ANCA + PR3/ MPO Ð

Repeat ANCA –

–

None (X-ray)

Mucosal thickening within maxillary antra. Mild deviation cartilaginous nasal septum with extensive septum defect. Minimal mucosal thickening within sphenoid sinus and ethmoid air cells.

–

C-ANCA + PR3/MPO Ð

Repeat ANCA –

–

None (X-ray)

–

Sinus biopsy: mesangial proliferative glomerulonephritis with crescents. IgA and fibrinogen deposition in mesangial matrix. Complement Deposition. Appearances suggestive of Henoch Schonlein Purpura.

Skin biopsy: florid leucocytoclastic vasculitis. Mild superficial perivascular predominantly neutrophilic inflammatory cell infiltrate with leucocytoclastic, extravasated erythrocytes.

P-ANCA + PR3 69

Repeat P-ANCA + PR3/MPO –

111

–

Pleural thickening (CT)

Concentric mucosal hypertrophy of maxillary antra with perforation in lower bony septum.

Sinus biopsy: Marked inflammatory change, densely inflamed granulation tissue with lymphocytes, neutrophils and plasma cells.

P-ANCA + PR3 + 4.2

Repeat P-ANCA + PR3/MPO –

Unconfirmed

–

Unremarkable appearances (CT)

Mucosal thickening within bilateral middle and inferior meati. Erosion of floor of nasal cavity, hard palate and nasal septum.

Nasal biopsy: inflammatory features with ulceration, no evidence of vasculitis, granuloma or malignancy. IgG4 plasma cells >80%. Sclerosing/eosinophilic angiocentric fibrosis noted.

PANCA + PR3/MPO Ð

Repeat P-ANCA + PR3/MPO –

Unconfirmed

–

Non-specific pulmonary nodules (CT)

Irregular thickening in nasal vestibule extending in to inferior meatus. Occlusive tissue in left middle meatus. No osseous or destructive lesion. Mild to moderate polyploid mucosal thickening of the maxillary antra and anterior ethmoid air cells.

Nasal biopsy: widespread mixed inflammatory infiltrate of neutrophils, lymphocytes, plasma cells and macrophages. No significant eosinophil infiltrate. Vascular necrosis and inflammation nasal ulceration.

P-ANCA + PR3 + 22.0, MPO Ð

Repeat P-ANCA + PR3 + 16, MPO –

–

None (X-Ray)

Patchy mucosal thickening within maxillary and ethmoid sinuses bilaterally, Nasal septal thickening of left middle/inferior turbinates. Perforation anteriorly and crowding of left nasal cavity

Nasal biopsy: fibrin slough with dense neutrophil infiltrate. Acute inflammation.

P-ANCA + PR3 + 18 MPO Ð

Repeat P-ANCA + PR3 + 15, MPO –

–

None (X-Ray)

Sinonasal mucosal thickening, obstruction of left ostiomental complex. Bony erosion of the sphenoid sinus floor with bony sclerosis.

–

Table 1: Demographics, serology and organ involvement of eleven patients with ANCA-associated vasculitis induced by cocaine. ESR Ð Erythrocyte Sedimentation Rate, CRP Ð C-Reactive Protein, PNS Ð Peripheral Nervous System, ENT Ð Ear, Nose and Throat, CT Ð Computerised Tomography, C-ANCA – cytoplasmic anti-neutrophil cytoplasmic antibody, P-ANCA – perinuclear anti-neutrophil cytoplasmic antibody, MPO Ð myeloperoxidase, PR3 – proteinase 3.

Disclosure: S. Subesinghe, None; S. van Leuven, None; L. Yalakki, None; S. Sangle (Joint First Author), None; D. P. D'Cruz, None.

To cite this abstract in AMA style:

Subesinghe S, van Leuven S, Yalakki L, Sangle (Joint First Author) S, D'Cruz DP. Cocaine and ANCA Associated Vasculitis-like Syndromes – a Case Series [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/cocaine-and-anca-associated-vasculitis-like-syndromes-a-case-series/. Accessed .

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