The renal and neurological complications of the vasculitides, paraproteinaemias, and granulomatous conditions
| Disease | Pathological features | Renal | Neurological |
|---|---|---|---|
| Vasculitides: | |||
| Primary | |||
| Polyarteritis nodosa | Necrotising vasculitis of medium and small vessels | 70% show proteinuria and granular casts progressing to renal failure. 50% have hypertension | 60% have peripheral neuropathy - most commonly painful mononeuropathy. 40% have CNS involvement with encephalopathy, focal infarction, subarachnoid haemorrhage, seizures and cranial neuropathies |
| Churg-Strauss angiitis | Eosinophilic necrotising vaculitis of medium and small vessels, peripheral eosinophilia | Infrequent renal involvement, rarely granular casts and hypertension | Mononeuropathy multiplex in 75%. Central nervous system involvement in 15-20% manifesting as encephalopathy, subarachnoid haemorrhage, rarely chorea |
| Wegener’s granulomatosis | Necrotising granulomatous vasculitis affecting respiratory tract and small vessels. Crescentic glomerulonephritis | Proteinuria, haematuria, red blood cell casts, culminating in renal failure | Cranial neuropathies due to local erosion by sinus granuloma. Multiple mononeuropathy and polyneuropathy, rarely focal CNS ischaemia |
| Secondary: | |||
| Infections | Bacterial and viral eg hepatitis B associated PAN | ||
| Toxins | Commonly in relation to illicit drug use | Proteinuria, granular casts culminating in renal failure | Encephalopathy, focal infarction in the central nervous system, mononeuropathy |
| Neoplasia | Commonly lymphoid malignancy | ||
| Connective tissue diseases: | |||
| Rheumatoid arthritis | Polyarthritis with synovial hypertrophy. Vasculitis of small and medium sized arteries | Rarely glomerulonephritis. Possible association with amyloidosis | Sensory or sensory motor peripheral neuropathy. Rarely mononeuropathy. Rare ischaemic central nervous system damage. Cranio-vertebral junction and high cervical cerd lesions in association with atlantoaxial subluxation and pannus formation |
| Systemic lupus erythematosus | Immune complex deposition and direct autoantibody effects | Haematuria, proteinuria, nephrotic syndrome, renal failure | Encephalopathy in 40% including neuropsychiatric and behavioural abnormalities. Seizures as presenting symptom in 5%. Cerebrovascular accidents, chorea, cranial neuropathies. Rarely distal sensory or sensory motor neuropathy and occasionally chronic inflammatory demyelinating polyneuropathy |
| Sjögren’s disease | Commonly presence of anti-Ro and anti-La antibodies | Lymphoid infiltration, tubular disorders and failure of acidification of urine | Peripheral neuropathy. Dorsal root ganglioneuropathy. Autonomic neuropathy. Cranial neuropathy seen in 40%. Psychiatric disorders and focal central nervous system disturbances which may mimic multiple sclerosis |
| Plasma cell dyscrasias | |||
| Multiple myeloma | Tissue infiltration with plasma cells. Direct effect of antibodies | Proteinuria (Bence-Jones), nephrotic syndrome, chronic renal failure | Nerve root and spinal cord compression. Intracranial cerebral and cranial nerve compression. Peripheral neuropathy - relatively rare, usually axonal and sensorimotor |
| POEMS (Osteosclerotic myeloma) | Binding of immunoglobulins to neural components. Cytokine effects | M-protein rarely discovered in urine. Proteinuria uncommon. Haemangiomas may occur in the kidney | 50% of patients have predominantly motor neuropathy resembling chronic inflammatory demyelinating polyneuropathy (CIDP) |
| Monoclonal gammopathies of unknown significance (MGUS) | Associated with lymphoid and non-lymphoid neoplasia and other autoimmune conditions. Probably affects 3% of the population | Rarely significant abnormality. Occasionally proteinuria and rarely amyloid deposition | Progressive sensory > motor demyelinating neuropathy (IgM), CIDP |
| Waldenström’s macroglobulinaemia | Uncontrolled proliferation of lymphcytes and plasma cells | Proteinuria, nephrotic syndrome and renal failure | Slowly progressive sensory and motor neuropathy. Encephalopathy due to hyperviscosity syndrome. Myelopathy, cerebrovascular accidents and subarachnoid haemorrhage |
| Cryoglobulinaemia | |||
| Type 1 (single monoclonal protein) | Waldenström’s macroglobulinaemia, multiple myeloma, lymphoproliferative disease | See above | See above |
| Type 2 (monoclonal IgM rheumatoid factor and polyclonal IgG) | Chronic infections. | Renal failure (glomerulonephritis), nephrotic syndrome | Multiple mononeuropathy, sensory and motor neuropathy in 7%. Transient ischaemic attacks, cerebral infarction |
| Type 3 ( polyclonal IgM rheumatoid factor and polyclonal IgG) | Chronic inflammatory or infective processes. Mixed essential cryoglobulinaemia |