Clinical Cases

Clinical context: The patient presented with renal failure and nephritic syndrome, and kidney biopsy confirmed rapidly progressive glomerulonephritis in the setting of MPO-ANCA vasculitis.

Capillaroscopy: Capillaroscopy shows mildly reduced density (7.0 capillaries/mm), frequent capillary dilations, hemorrhages, and no giant capillaries, consistent with a non-specific microvascular pattern.

Clinical context: After 3 months of progressive symptoms, diagnosis was established based on proximal muscle weakness, Gottron papules, heliotrope rash, and hyperCKemia (~2500).

Capillaroscopy: There is marked capillary loss (4.8/mm), architectural disorganization, frequent dilations, giant capillaries, and abnormal/branched shapes, indicating severe active microangiopathy.

Clinical context: This case includes a 5-year history of Raynaud's phenomenon, esophageal dysmotility, and interstitial lung disease with an NSIP pattern, without current indirect echocardiographic signs of pulmonary hypertension.

Capillaroscopy: Capillaroscopy shows marked capillary loss (5.3/mm), frequent dilations, high prevalence of giant capillaries (27.5%), and hemorrhages, matching an active Cutolo pattern.

Clinical context: The case includes a 10-year history of acrocyanosis with tingling and burning sensations, more pronounced in cold environments. He has mild esophageal dysmotility and several apical subpleural fibrous nodules on chest CT, without current indirect echocardiographic signs of pulmonary hypertension. He is currently being treated with mycophenolate mofetil and amlodipine.

Capillaroscopy: Capillaroscopy shows marked capillary reduction (5.86/mm), frequent dilations (54%), a high prevalence of giant capillaries (39.7%), and hemorrhages, without avascular areas, matching an active Cutolo pattern. The CSURI index has increased. Blood flow is homogeneous, with reduced linear velocity.

Clinical context: Patient under follow-up by internal medicine, in whom a positive ANA was documented, with findings compatible with nonspecific interstitial pneumonia. The patient had an ANA titer greater than 1:1280 with an AC-9 granular pattern. Subsequent studies showed high-titer positivity for anti-U3 RNP antibodies. The patient presented with salt-and-pepper pigmentation, generalized cutaneous sclerosis, and sclerodactyly.

Capillaroscopy: There is marked capillary loss (4.9/mm), architectural disorganization, frequent dilations, giant capillaries in 14.9% of capillaries, and abnormal or ramified forms. This pattern was classified by the CAPI-Detect algorithm as active; however, due to the frequent features characteristic of late-stage involvement, it was ultimately classified as such in the final report.

Clinical context: The patient developed progressive proximal weakness, dysphagia, heliotrope erythema, malar and facial erythema, and scaly cutaneous lesions, with CK elevation up to 2503 U/L and a positive myositis panel for anti-NXP2 antibodies. After corticosteroid and immunosuppressive treatment, she improved clinically and analytically.

Capillaroscopy: The first study shows an active scleroderma/myositis-like microangiopathy with severe capillary loss, giant capillaries, arborizing ramifications, hemorrhages, architectural disorganization, and avascular areas. The follow-up study shows regression to a non-specific pattern with improved density and disappearance of giant capillaries and hemorrhages.

Clinical context: Two-year history of V-neck lesions and hypomyopathic weakness on chronic low-dose methotrexate and prednisone.

Capillaroscopy: Capillaroscopy shows severely reduced capillary density (2.7/mm), architectural disorganization, frequent dilations, giant capillaries, and abnormal/ramified forms (2/3 on the semi-quantitative scale), consistent with a scleroderma-like pattern.

Clinical context: The patient was referred to rheumatology because of skin lesions compatible with juvenile dermatomyositis. Examination showed Gottron papules on the fingers and Gottron signs on the ankles, elbows, and knees. She had no muscle weakness, with an MMT score of 80/80. Laboratory testing showed normal CPK, positive ANA, and weak anti-PM/Scl positivity, with the remaining autoantibodies negative. Treatment was started with hydroxychloroquine and intravenous immunoglobulin.

Capillaroscopy: The baseline capillaroscopy is compatible with juvenile dermatomyositis and shows a severe scleroderma-like microangiopathy, with marked capillary loss, frequent enlarged and giant capillaries, hemorrhages, abnormal or arborizing shapes, and architectural disorganization.

Clinical context: The patient presented with several weeks of shoulder and pelvic girdle weakness, without skin involvement or other symptoms. Laboratory testing showed CPK elevation up to 8000 U/L, lymphopenia, thrombocytopenia, and low-titer positive ANA. Whole-body MRI showed muscle edema in the girdles. The initial myositis panel was negative, so the early scleroderma-like capillaroscopy pattern helped support the diagnosis of juvenile dermatomyositis despite the absence of skin disease or specific autoantibodies. An extended panel later detected anti-NXP2 antibodies. Treatment was started with hydroxychloroquine, mycophenolate mofetil, and intravenous immunoglobulin, followed by clinical improvement and CK reduction.

Capillaroscopy: The June 2024 capillaroscopy showed an early scleroderma-like pattern compatible with juvenile dermatomyositis, including reduced density, frequent enlarged capillaries, sparse giant capillaries, and hemorrhages. The December 2024 follow-up showed disappearance of giant capillaries and evolution toward a non-specific pattern, with improved capillary density.

Clinical context: The patient was referred for skin lesions, including Gottron lesions and facial erythema, together with muscle weakness and dysphagia. Complementary tests were compatible with juvenile dermatomyositis, and anti-TIF1 antibodies were positive.

Capillaroscopy: Capillaroscopy shows a severe active scleroderma-like pattern, with very low capillary density, numerous giant capillaries, frequent enlarged capillaries, arborizing abnormal shapes, and architectural disorganization. This morphology is highly suggestive of active microangiopathy in juvenile dermatomyositis.

Clinical context: The patient presented with a 3-month history of Raynaud phenomenon. Her family history included a father with systemic lupus erythematosus. Examination showed swelling of the fingers and delayed capillary refill. Laboratory testing showed anti-RNP positivity. Months later she developed carpal arthritis, leading to a diagnosis of mixed connective tissue disease.

Capillaroscopy: The initial capillaroscopy showed an early scleroderma pattern, supporting the diagnosis in the appropriate clinical and serological context. On follow-up, clinical improvement was accompanied by improvement of capillaroscopic findings, with higher density, disappearance of giant capillaries, and evolution toward a non-specific pattern.